OrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesDisciplines and OccupationsInformation Science
Complement C3calpha-GalactosidaseFabry DiseaseGalactosidasesbeta-GalactosidaseCerebrosidesLactoseGalactoseAcridine Orangealpha-L-FucosidaseNigericinGlycoside HydrolasesGlycosidesCell AgingHistocytochemistryEscherichia colialpha 1-AntitrypsinMolecular Sequence DataReceptors, Adrenergic, alphaHypoxia-Inducible Factor 1, alpha Subunitalpha7 Nicotinic Acetylcholine ReceptorIntegrin alpha3beta1Integrin alpha4Integrin alpha6Integrin alpha5beta1Integrin alpha4beta1Interleukin-1alphaIntegrin alpha2beta1Receptors, Adrenergic, alpha-1
Complement C3c
A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.
alpha-Galactosidase
An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids.
Fabry Disease
An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.
Galactosidases
A family of galactoside hydrolases that hydrolyze compounds with an O-galactosyl linkage. EC 3.2.1.-.
beta-Galactosidase
A group of enzymes that catalyzes the hydrolysis of terminal, non-reducing beta-D-galactose residues in beta-galactosides. Deficiency of beta-Galactosidase A1 may cause GANGLIOSIDOSIS, GM1.
Cerebrosides
Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)
Lactose
A disaccharide of GLUCOSE and GALACTOSE in human and cow milk. It is used in pharmacy for tablets, in medicine as a nutrient, and in industry.
Galactose
An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.
Acridine Orange
A cationic cytochemical stain specific for cell nuclei, especially DNA. It is used as a supravital stain and in fluorescence cytochemistry. It may cause mutations in microorganisms.
alpha-L-Fucosidase
An enzyme that catalyzes the hydrolysis of an alpha L-fucoside to yield an alcohol and L-fucose. Deficiency of this enzyme can cause FUCOSIDOSIS. EC 3.2.1.51.
Nigericin
A polyether antibiotic which affects ion transport and ATPase activity in mitochondria. It is produced by Streptomyces hygroscopicus. (From Merck Index, 11th ed)
Glycoside Hydrolases
Glycosides
Any compound that contains a constituent sugar, in which the hydroxyl group attached to the first carbon is substituted by an alcoholic, phenolic, or other group. They are named specifically for the sugar contained, such as glucoside (glucose), pentoside (pentose), fructoside (fructose), etc. Upon hydrolysis, a sugar and nonsugar component (aglycone) are formed. (From Dorland, 28th ed; From Miall's Dictionary of Chemistry, 5th ed)
Cell Aging
The decrease in the cell's ability to proliferate with the passing of time. Each cell is programmed for a certain number of cell divisions and at the end of that time proliferation halts. The cell enters a quiescent state after which it experiences CELL DEATH via the process of APOPTOSIS.
Histocytochemistry
Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.
Escherichia coli
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
alpha 1-Antitrypsin
Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Receptors, Adrenergic, alpha
One of the two major pharmacological subdivisions of adrenergic receptors that were originally defined by the relative potencies of various adrenergic compounds. The alpha receptors were initially described as excitatory receptors that post-junctionally stimulate SMOOTH MUSCLE contraction. However, further analysis has revealed a more complex picture involving several alpha receptor subtypes and their involvement in feedback regulation.
Hypoxia-Inducible Factor 1, alpha Subunit
Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by OXYGEN availability and is targeted for degradation by VHL TUMOR SUPPRESSOR PROTEIN.
alpha7 Nicotinic Acetylcholine Receptor
A member of the NICOTINIC ACETYLCHOLINE RECEPTOR subfamily of the LIGAND-GATED ION CHANNEL family. It consists entirely of pentameric a7 subunits expressed in the CNS, autonomic nervous system, vascular system, lymphocytes and spleen.
Integrin alpha3beta1
Cell surface receptor for LAMININ, epiligrin, FIBRONECTINS, entactin, and COLLAGEN. Integrin alpha3beta1 is the major integrin present in EPITHELIAL CELLS, where it plays a role in the assembly of BASEMENT MEMBRANE as well as in cell migration, and may regulate the functions of other integrins. Two alternatively spliced isoforms of the alpha subunit (INTEGRIN ALPHA3), are differentially expressed in different cell types.
Integrin alpha4
An integrin alpha subunit that is unique in that it does not contain an I domain, and its proteolytic cleavage site is near the middle of the extracellular portion of the polypeptide rather than close to the membrane as in other integrin alpha subunits.
Integrin alpha6
An integrin alpha subunit that primarily associates with INTEGRIN BETA1 or INTEGRIN BETA4 to form laminin-binding heterodimers. Integrin alpha6 has two alternatively spliced isoforms: integrin alpha6A and integrin alpha6B, which differ in their cytoplasmic domains and are regulated in a tissue-specific and developmental stage-specific manner.
Integrin alpha5beta1
An integrin found in FIBROBLASTS; PLATELETS; MONOCYTES, and LYMPHOCYTES. Integrin alpha5beta1 is the classical receptor for FIBRONECTIN, but it also functions as a receptor for LAMININ and several other EXTRACELLULAR MATRIX PROTEINS.
Integrin alpha4beta1
Integrin alpha4beta1 is a FIBRONECTIN and VCAM-1 receptor present on LYMPHOCYTES; MONOCYTES; EOSINOPHILS; NK CELLS and thymocytes. It is involved in both cell-cell and cell- EXTRACELLULAR MATRIX adhesion and plays a role in INFLAMMATION, hematopoietic cell homing and immune function, and has been implicated in skeletal MYOGENESIS; NEURAL CREST migration and proliferation, lymphocyte maturation and morphogenesis of the PLACENTA and HEART.
Interleukin-1alpha
An interleukin-1 subtype that occurs as a membrane-bound pro-protein form that is cleaved by proteases to form a secreted mature form. Unlike INTERLEUKIN-1BETA both membrane-bound and secreted forms of interleukin-1alpha are biologically active.
Integrin alpha2beta1
An integrin found on fibroblasts, platelets, endothelial and epithelial cells, and lymphocytes where it functions as a receptor for COLLAGEN and LAMININ. Although originally referred to as the collagen receptor, it is one of several receptors for collagen. Ligand binding to integrin alpha2beta1 triggers a cascade of intracellular signaling, including activation of p38 MAP kinase.
Receptors, Adrenergic, alpha-1
A subclass of alpha-adrenergic receptors that mediate contraction of SMOOTH MUSCLE in a variety of tissues such as ARTERIOLES; VEINS; and the UTERUS. They are usually found on postsynaptic membranes and signal through GQ-G11 G-PROTEINS.

Lignocellulose degradation by Phanerochaete chrysosporium: purification and characterization of the main alpha-galactosidase. (1/449)

The main alpha-galactosidase was purified to homogeneity, in 30% yield, from a solid culture of Phanerochaete chrysosporium on 1 part wheat bran/2 parts thermomechanical softwood pulp. It is a glycosylated tetramer of 50 kDa peptide chains, which gives the N-terminal sequence ADNGLAITPQMG(?W)NT(?W)NHFG(?W)DIS(?W)DTI. It is remarkably stable, with crude extracts losing no activity over 3 h at 80 degrees C, and the purified enzyme retaining its activity over several months at 4 degrees C. The kinetics of hydrolysis at 25 degrees C of various substrates by this retaining enzyme were measured, absolute parameters being obtained by active-site titration with 2',4',6'-trinitrophenyl 2-deoxy-2, 2-difluoro-alpha-D-galactopyranoside. The variation of kcat/Km for 1-naphthyl-alpha-D-galactopyranoside with pH is bell-shaped, with pK1=1.91 and pK2=5.54. The alphaD(V/K) value for p-nitrophenyl-alpha-D-glucopyranoside is 1.031+/-0.007 at the optimal pH of 3.75 and 1.114+/-0.006 at pH7.00, indicating masking of the intrinsic effect at optimal pH. There is no alpha-2H effect on binding galactose [alphaD(Ki)=0.994+/-0.013]. The enzyme hydrolyses p-nitrophenyl beta-L-arabinopyranoside approximately 510 times slower than the galactoside, but has no detectable activity on the alpha-D-glucopyranoside or alpha-D-mannopyranoside. Hydrolysis of alpha-galactosides with poor leaving groups is Michaelian, but that of substrates with good leaving groups exhibits pronounced apparent substrate inhibition, with Kis values similar to Km values. We attribute this to the binding of the second substrate molecule to a beta-galactopyranosyl-enzyme intermediate, forming an E.betaGal. alphaGalX complex which turns over slowly, if at all. 1-Fluoro-alpha-D-galactopyranosyl fluoride, unlike alpha-D-galactopyranosyl fluoride, is a Michaelian substrate, indicating that the effect of 1-fluorine substitution is greater on the first than on the second step of the enzyme reaction.  (+info)

Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. (2/449)

Fabry disease is an X-linked metabolic disorder caused by a deficiency of alpha-galactosidase A (alpha-Gal A). The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide (Gb3). In patients with this disorder, glycolipid deposition in endothelial cells leads to renal failure and cardiac and cerebrovascular disease. Recently, we generated alpha-Gal A gene knockout mouse lines and described the phenotype of 10-week-old mice. In the present study, we characterize the progression of the disease with aging and explore the effects of bone marrow transplantation (BMT) on the phenotype. Histopathological analysis of alpha-Gal A -/0 mice revealed subclinical lesions in the Kupffer cells in the liver and macrophages in the skin with no gross lesions in the endothelial cells. Gb3 accumulation and pathological lesions in the affected organs increased with age. Treatment with BMT from the wild-type mice resulted in the clearance of accumulated Gb3 in the liver, spleen, and heart with concomitant elevation of alpha-Gal A activity. These findings suggest that BMT may have a potential role in the management of patients with Fabry disease.  (+info)

Differential expression of three alpha-galactosidase genes and a single beta-galactosidase gene from Aspergillus niger. (3/449)

A gene encoding a third alpha-galactosidase (AglB) from Aspergillus niger has been cloned and sequenced. The gene consists of an open reading frame of 1,750 bp containing six introns. The gene encodes a protein of 443 amino acids which contains a eukaryotic signal sequence of 16 amino acids and seven putative N-glycosylation sites. The mature protein has a calculated molecular mass of 48,835 Da and a predicted pI of 4.6. An alignment of the AglB amino acid sequence with those of other alpha-galactosidases revealed that it belongs to a subfamily of alpha-galactosidases that also includes A. niger AglA. A. niger AglC belongs to a different subfamily that consists mainly of prokaryotic alpha-galactosidases. The expression of aglA, aglB, aglC, and lacA, the latter of which encodes an A. niger beta-galactosidase, has been studied by using a number of monomeric, oligomeric, and polymeric compounds as growth substrates. Expression of aglA is only detected on galactose and galactose-containing oligomers and polymers. The aglB gene is expressed on all of the carbon sources tested, including glucose. Elevated expression was observed on xylan, which could be assigned to regulation via XlnR, the xylanolytic transcriptional activator. Expression of aglC was only observed on glucose, fructose, and combinations of glucose with xylose and galactose. High expression of lacA was detected on arabinose, xylose, xylan, and pectin. Similar to aglB, the expression on xylose and xylan can be assigned to regulation via XlnR. All four genes have distinct expression patterns which seem to mirror the natural substrates of the encoded proteins.  (+info)

Molecular cloning of a human UDP-galactose:GlcNAcbeta1,3GalNAc beta1, 3 galactosyltransferase gene encoding an O-linked core3-elongation enzyme. (4/449)

Using the full-length amino-acid sequences of the human beta1,3 galactosyltransferase (beta3GalT)-I, -II and III enzymes as query, we have identified an additional member of the beta3GalT gene family within a sequenced region of the human chromosome 21 as found in GenBank. The novel human beta3GalT-V gene included an open reading frame of 933 bp encoding a protein of 310 amino acids with a short N-terminal cytoplasmic tail, a single predicted transmembrane domain and a large lumenal catalytic domain. The human beta3GalT-V protein showed 34%, 27%, 31% and 23% sequence identity with the human beta3GalT-I, -II, -III and -IV enzymes, respectively. The expression of beta3GalT-V as a recombinant protein in Sf9 insect cells confirmed the galactosyltransferase activity catalyzed by this enzyme. Similarly to beta3GalT-I, -II and -III, the beta3GalT-V enzyme used beta-linked GlcNAc as an acceptor, but unlike the former enzymes beta3GalT-V exhibited a marked preference for the O-linked core3 GlcNAcbeta1,3GalNAc substrate. The beta3GalT-V gene was mainly expressed in human small intestine and to a lesser extent in pancreas and testis. Although beta3GalT-V transcripts were not detected in normal colon tissue, based on Northern analysis, beta3GalT-V mRNA was found in the adenocarcinoma cell line Colo 205.  (+info)

Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome. (5/449)

A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.  (+info)

Purification and characterization of recombinant Mortierella vinacea alpha-galactosidases I and II expressed in Saccharomyces cerevisiae. (6/449)

The cDNAs coding for Mortierella vinacea alpha-galactosidases I and II were expressed in Saccharomyces cerevisiae under the control of the yeast GAL10 promoter. The recombinant enzymes purified to homogeneity from the culture filtrate were glycosylated, and had properties identical to those of the native enzymes except for improving the heat stability of alpha-galactosidase II and decreasing the specific activities of both enzymes.  (+info)

Cloning of the gene encoding a novel thermostable alpha-galactosidase from Thermus brockianus ITI360. (7/449)

An alpha-galactosidase gene from Thermus brockianus ITI360 was cloned, sequenced, and expressed in Escherichia coli, and the recombinant protein was purified. The gene, designated agaT, codes for a 476-residue polypeptide with a calculated molecular mass of 53, 810 Da. The native structure of the recombinant enzyme (AgaT) was estimated to be a tetramer. AgaT displays amino acid sequence similarity to the alpha-galactosidases of Thermotoga neapolitana and Thermotoga maritima and a low-level sequence similarity to alpha-galactosidases of family 36 in the classification of glycosyl hydrolases. The enzyme is thermostable, with a temperature optimum of activity at 93 degrees C with para-nitrophenyl-alpha-galactopyranoside as a substrate. Half-lives of inactivation at 92 and 80 degrees C are 100 min and 17 h, respectively. The pH optimum is between 5.5 and 6.5. The enzyme displayed high affinity for oligomeric substrates. The K(m)s for melibiose and raffinose at 80 degrees C were determined as 4.1 and 11.0 mM, respectively. The alpha-galactosidase gene in T. brockianus ITI360 was inactivated by integrational mutagenesis. Consequently, no alpha-galactosidase activity was detectable in crude extracts of the mutant strain, and it was unable to use melibiose or raffinose as a single carbohydrate source.  (+info)

Gene assignment in the spider monkey (Ateles paniscus chamek--APC): APE-MYH7 to 2q; AR-GLA-F8C to the X chromosome. (8/449)

Comparative gene assignment between the spider monkey species Ateles paniscus chamek (APC) and man (HSA) showed conserved syntenic associations despite extensive karyotypic rearrangement between species. Two HSA 14q genes were allocated to APC 2q, being syntenic to other HSA 14q and HSA 15q markers previously assigned to APC 2q, and to HSA 12q genes previously assigned to APC 2p. These findings were consistent with A. geoffroyi chromosome painting with human whole-chromosome probes, indicating that the genus Ateles is karyotypically very rearranged. On the other hand, three human X-linked markers were assigned to the Ateles X chromosome, indicating that this chromosome is evolutionary stable.  (+info)

جامعة الأندلس الخاصة للعلوم الطبية - Agalsidase Beta (Fabrazyme)جامعة الأندلس الخاصة للعلوم الطبية - Agalsidase Beta (Fabrazyme)
Ninety-five of 121 (79%) adult patients and 11 of 16 (69%) pediatric patients (106 of 137, 74% of all patients) treated with Fabrazyme (agalsidase beta) in clinical studies have developed IgG antibodies to Fabrazyme (agalsidase beta) . Most patients who develop IgG antibodies do so within the first 3 months of exposure. IgG seroconversion in pediatric patients was associated with prolonged half-life of Fabrazyme (agalsidase beta) , a phenomenon rarely observed in adult patients [see CLINICAL PHARMACOLOGY and Use in Specific Populations]. A possible cause for this prolongation likely pertains to the ability of antibodies to act as carriers for their antigens. Among the 14 female patients exposed to Fabrazyme (agalsidase beta) in clinical studies, four (two adult and two pediatric patients) developed IgG antibodies to Fabrazyme (agalsidase beta) . IgG antibodies to Fabrazyme (agalsidase beta) were purified from 15 patients with high antibody titers ( ≥ 12,800) and studied for inhibition of in ...
https://au.edu.sy/faculties/pharmacy-faculty/drug-encyclopedia/1633-a/6177-agalsidase-beta-fabrazyme
agalsidase beta - WellSpan Health Libraryagalsidase beta - WellSpan Health Library
Agalsidase beta is a man-made form of the naturally-occurring alpha-galactosidase A enzyme. A deficiency of this enzyme is called Fabry disease. Agalsidase beta reduces deposits of globotriaosylceramide (GL-3) in the kidneys and certain other cells in the body. Agalsidase beta is used in the treatment of Fabry disease...
https://www.wellspan.org/health-library/Document.aspx?id=d04866a1
Immobilization and stabilization of alpha-galactosidase on Sepabeads EC-EA and EC-HA | AVESİSImmobilization and stabilization of alpha-galactosidase on Sepabeads EC-EA and EC-HA | AVESİS
alpha-Galactosidase from tomato has been immobilized on Sepabead EC-EA and Sepabead EC-HA, which were activated with ethylendiamino and hexamethylenediamino groups, respectively. Two strategy was used for the covalent immobilization of alpha-galactosidase on the aminated Sepabeads: covalent immobilization of enzyme on glutaraldehyde activated support and cross-linking of the adsorbed enzymes on to the support with glutaraldehyde. By using these two methods, all the immobilized enzymes retained very high activity and the stability of the enzyme was also improved. The obtained results showed that, the most stable immobilized alpha-galactosidase was obtained with the second strategy. The immobilized enzymes were characterized with respect to free counterpart. Some parameters effecting to the enzyme activity and stability were also analyzed. The optimum temperature and pH were found as 60 degrees C and pH 5.5 for all immobilized enzymes, respectively. All the immobilized alpha-galactosidases were ...
https://avesis.istanbul.edu.tr/yayin/d5b04226-7c3c-466d-b7ba-aa46fd6e4b93/immobilization-and-stabilization-of-alpha-galactosidase-on-sepabeads-ec-ea-and-ec-ha
Safety and Efficacy of PRX 102 in Patients With Fabry Disease Currently Treated With REPLAGAL® (Agalsidase Alfa) - Full Text...Safety and Efficacy of PRX 102 in Patients With Fabry Disease Currently Treated With REPLAGAL® (Agalsidase Alfa) - Full Text...
This is an open label switch over study to assess the safety and efficacy of PRX-102 (pegunigalsidase alfa). Patients treated with agalsidase alfa for at least 2 years and on a stable dose (,80% labelled dose/kg) for at least 6 months. Patients will be screened and evaluated over 3 months while continuing on agalsidase alfa. Following the screening period, the patient will be enrolled and switched from their agalsidase alfa treatment to receive intravenous (IV) infusions of PRX-102 1 mg/kg every two weeks for 12 months. No more than 25% of treated patients will be female ...
https://www.clinicaltrials.gov/ct2/show/NCT03018730
Keywords: agalsidase alfa efficacy enzyme replacement therapy SPTAN1 - CFTR Inhibitors for Treating Diarrheal DiseaseKeywords: agalsidase alfa efficacy enzyme replacement therapy SPTAN1 - CFTR Inhibitors for Treating Diarrheal Disease
CGS-15943 (SD) baseline SDNN 81.6 (20.9) ms; mean (95% confidence interval [CI]) change from baseline to week 55 17.4 (2.9 31.9 ms. Changes in SDNN correlated with changes in LVMI (r=?0.975). No change occurred in secondary efficacy endpoints: mean (95% CI) change from baseline at week 55 in LVMI 0.16 (?3.3 3.7 g/m2.7; midwall fractional shortening ?0.62% (?2.7% 1.5%); estimated glomerular filtration rate 0.15 (?11.4 11.7 mL/min/1.73 m2; urine protein ?1.8 (?6.0 2.4 mg/dL; urine microalbumin 0.6 (?0.5 1.7 mg/dL; plasma globotriaosylceramide (Gb3) ?5.71 (?10.8 ?0.6) nmol/mL; urinary Gb3 ?1 403.3 (?3 714 907.4 nmol/g creatinine or clinical quality-of-life outcomes. Conclusion Fifty-five weeks agalsidase alfa ERT at 0.2 mg/kg every other week was well tolerated. Disease progression may be slowed when ERT is started prior to major organ dysfunction. CGS-15943 Trial registration https://ClinicalTrials.gov identifier NCT01363492. Keywords: agalsidase alfa efficacy enzyme replacement therapy SPTAN1 ...
https://teannualconference2015.info/tag/keywords-agalsidase-alfa-efficacy-enzyme-replacement-therapy-sptan1/
alpha-Galactosidase 4A from Bacillus halodurans | | Apollo Scientificalpha-Galactosidase 4A from Bacillus halodurans | | Apollo Scientific
Product Name:alpha-Galactosidase 4A from Bacillus halodurans CAS Number: Catalouge Number:BIZY1000 Purity: Commodity Code:99999999 MDL Number: Notes:alpha-Galactosidases are enzymes that participate in the hydrolysis of terminal, non-reducing alpha-D-galactose. Family: GH4. E.C.:3.2.1.22. Architecture: GH4. Main activity:alpha-Galactosidase. Synonyms:
https://store.apolloscientific.co.uk/product/alpha-galactosidase-4a-from-bacillus-halodurans
Globotriaosylceramide - WikipediaGlobotriaosylceramide - Wikipedia
Defects in the enzyme alpha-galactosidase lead to the buildup of globotriaosylceramide, causing Fabrys disease.[3] The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabrys. Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC). The bacterial Shiga toxin can be used for targeted therapy of gastric cancer, because this tumor entity expresses the receptor of the Shiga toxin. For this purpose an unspecific chemotherapeutical is conjugated to the B-subunit to make it specific. In this way only the tumor cells, but not healthy cells should be destroyed during therapy.[4] ...
https://en.wikipedia.org/wiki/Globotriaosylceramide
Fabrazyme (Agalsidase Beta): Side Effects, Interactions, Warning, Dosage & UsesFabrazyme (Agalsidase Beta): Side Effects, Interactions, Warning, Dosage & Uses
Learn about Fabrazyme (Agalsidase Beta) may treat, uses, dosage, side effects, drug interactions, warnings, patient labeling, reviews, and related medications.
https://www.rxlist.com/fabrazyme-drug.htm
Evaluation of a low dose, after a standard therapeutic dose, of agalsidase beta during enzyme replacement therapy in patients...Evaluation of a low dose, after a standard therapeutic dose, of agalsidase beta during enzyme replacement therapy in patients...
A lower dose of agalsidase beta may be sufficient in some, but not all, patients with Fabry disease to maintain the cellular globotriaosylceramide clearance achieved with 1.0 mg/kg/2 weeks. Long-term clinical effects of transitioning to the lower dose have not been evaluated.
https://pubmed.ncbi.nlm.nih.gov/19265719/?dopt=Abstract
Molecular pathology of Fabrys disease. Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial...Molecular pathology of Fabrys disease. Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial...
Semantic Scholar extracted view of Molecular pathology of Fabrys disease. Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells. by Douglas L. Johnson et al.
https://www.semanticscholar.org/paper/Molecular-pathology-of-Fabry-s-disease-Physical-an-Johnson-Desnick/a53581683e66173c726f7c82b33c61879fe7b7a3
English (en)English (en)
Atypical hemolytic-uremic syndrome (aHUS) is a rare, potentially lethal systemic disorder, capable of affecting both adults and children, causing thrombotic microangiopathy (TMA) that leads to the formation of thrombus within small blood vessels with multiple organ failure. The pathogenesis of the aHUS is part of a sort of chronic and uncontrolled activation of the complement system by genetic mutation of some proteins usually responsible for its self-regulation. Today, the rapid diagnosis of the disease and the timely start of treatment with eculizumab, improve outcomes of renal failure, stroke and heart attack. Fabry disease is a rare tesaurismosis, X linked, due to the deficiency of the lysosomal enzyme alpha-galactosidase A, necessary for the physiological catabolism of glycosphingolipids. Multisystem clinical manifestations lead to a serious degenerative pathology. The diagnostic suspicion based on anamnesis and careful research of the symptoms and then confirmed by the enzymatic dosage of ...
https://www.raredis.org/archives/author/informer/page/2?lang=en
AGAW - Overview: Alpha-Galactosidase, LeukocytesAGAW - Overview: Alpha-Galactosidase, Leukocytes
AGAW : Diagnosis of Fabry disease in male patients   Verifying abnormal serum alpha-galactosidase results in male patients with a clinical presentation suggestive of Fabry disease
https://test.mayocliniclabs.com/test-catalog/Overview/606261
Human natural anti-alpha-galactosyl IgG. II. The specific recognition of alpha (1----3)-linked galactose residues. | Journal of...Human natural anti-alpha-galactosyl IgG. II. The specific recognition of alpha (1----3)-linked galactose residues. | Journal of...
A natural IgG antibody (anti-Gal) with alpha-galactosyl binding specificity has been found in large amounts (0.5 - 1.0% of serum IgG) in all individuals tested. It has been purified by affinity chromatography on a column of melibiose-Sepharose. In addition to its affinity for normal and pathological senescent human red cells, the antibody readily interacts with rabbit red blood cell (RRBC) glycolipids with alpha-galactosyl terminal residues. Two types (glycosidic linkages of 1----3 vs. 1----4) of rabbit red cells glycolipids with terminal alpha-galactosyl residues were tested for antibody binding. The antibody specifically bound to glycolipids with Gal alpha 1----3 terminal residues, and treatment of these glycolipids with alpha-galactosidase abolished binding. Hemagglutination inhibition studies with oligosaccharides of known structure also showed that the antibody binds specifically to glycoconjugates with an alpha 1----3 terminal galactose residue. Anti-Gal did not bind to a human B-active ...
https://rupress.org/jem/article/162/2/573/58823/Human-natural-anti-alpha-galactosyl-IgG-II-The
WO 2016/097324 A1 - Alpha-galactosidases And Uses Thereof - The Lens - Free & Open Patent and Scholarly SearchWO 2016/097324 A1 - Alpha-galactosidases And Uses Thereof - The Lens - Free & Open Patent and Scholarly Search
The Lens serves almost all the patents and scholarly work in the world as a free, open and secure digital public good, with user privacy a paramount focus.
https://www.lens.org/lens/patent/074-348-578-095-74X
MegazymeMegazyme
Purchase high purity enzyme alpha-Galactosidase (Aspergillus niger) for use in research, biochemical enzyme assays and in vitro diagnostic analysis.
https://www.megazyme.com/tellafriend/index/sArticle/461
Pfam: He PIG assoc
GF ID He_PIG_assoc #=GF AC PF10632.8 #=GF DE He_PIG associated, NEW1 domain of bacterial glycohydrolase #=GF AU Naumoff D, Coggill P #=GF SE Pfam-B_97991 (release 22.0) #=GF GA 25.00 25.00; #=GF TC 25.20 30.80; #=GF NC 22.50 23.40; #=GF BM hmmbuild HMM.ann SEED.ann #=GF SM hmmsearch -Z 26740544 -E 1000 --cpu 4 HMM pfamseq #=GF TP Domain #=GF RN [1] #=GF RM 15285616 #=GF RT [Phylogenetic analysis of alpha-galactosidases of the GH27 #=GF RT family]. #=GF RA Naumov DG; #=GF RL Mol Biol (Mosk). 2004;38:463-476. #=GF RN [2] #=GF RM 16131397 #=GF RT GH97 is a new family of glycoside hydrolases, which is related #=GF RT to the alpha-galactosidase superfamily. #=GF RA Naumoff DG; #=GF RL BMC Genomics. 2005;6:112. #=GF DR INTERPRO; IPR019599; #=GF CC The English-language version of the first reference can be found #=GF CC on pages 388-399 of the above. This domain has been named NEW1 #=GF CC but its actual function is not known. It is found on proteins #=GF CC which are bacterial galactosidases [1]. The ...
http://www.genome.jp/dbget-bin/www_bget?pfam:He_PIG_assoc
What is the genotype of the EZ Competent Cells? - QIAGENWhat is the genotype of the EZ Competent Cells? - QIAGEN
Expression of N-terminally deleted beta-galactosidase.This protein and LacZ alpha-peptide (encoded by the pDrive Cloning Vector) provide alpha-galactosidase activity. Cells transformed by pDrive Cloning Vector which does not contain a PCR product will express LacZ alpha-peptide, and will form blue colonies when grown in the presence of X-gal/IPTG. In contrast, cells transformed by pDrive Cloning Vector which does contain a PCR product will not express LacZ alpha-peptide, and will form white colonies. ...
https://www.qiagen.com/ge/resources/faq?id=a526375a-2cce-4a7d-94da-66cda72c5f89&lang=en
Fabrazyme: Uses, Taking, Side Effects, Warnings - Medicine.comFabrazyme: Uses, Taking, Side Effects, Warnings - Medicine.com
Learn about Fabrazyme: What is it used for, what you need to know before taking, important warnings and safety info, how to take, side effects and more...
https://www.medicine.com/drug/fabrazyme
Beano Drops (Alpha-Galactosidase) GlaxoSmithKlineBeano Drops (Alpha-Galactosidase) GlaxoSmithKline
Beano Drops information about active ingredients, pharmaceutical forms and doses by GlaxoSmithKline, Beano Drops indications, usages and related health products lists
https://drugs-about.com/drugs-b/beano-drops.html
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In that case, lead plaintiff Joseph Carik, who has a doctors prescription for Fabrazyme, claimed: By and through FDA consent, plaintiff has been banned interstate access to FDA-approved doses of Fabrazyme during a drug shortage created by Genzyme (a Sanofi company) an FDA licensee. Plaintiff is instead forcibly injected with a diluted, unregulated, unapproved dose of Fabrazyme because if the plaintiff refuses infusion of the unapproved dose, then the FDA licensee will withdraw any access and not provide future access to the drug until the shortage is over. The United States defendants have delegated all medical decisions during the shortage regarding plaintiffs disease, life, and health to the sole discretion and control of a corporation regulated by and through grant of an FDA license ...
https://www.courthousenews.com/death-by-drug-shortage-widow-says/
Discount: Full Spectrum Digest 90 Vegcaps (A70003) By Allergy Research Group - Back Pain InformationDiscount: Full Spectrum Digest 90 Vegcaps (A70003) By Allergy Research Group - Back Pain Information
Full Spectrum Digest is a full spectrum vegan enzyme blend, including Glutalytic, lactase, lipase, alpha-galactosidase, and amylase, to assist breakdown of gluten, gliadin, casein, whey, lactose, protein, fats, and carbohydrates.. ...
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Ultra Veggie Enzymes - EarthbarUltra Veggie Enzymes - Earthbar
[tabs][tab title=Ingredients]Plant Enzyme Blend 383 mg Amylase (3,500 DU) Glucoamylase (5.25 AGU) Lipase (350 FCCLU) Protease I pH 3.0 (15 S APU) Protease II pH 4.5 (20,000 HUT) Protease III pH 6.0 (35,000 HUT ) Cellulase (200 CU) CereCalaseT M (250 MU) Alpha-Galactosidase (75 GaIU) Lactase (150 ALU) Invertase (150
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GLA gene homepage - Global Variome shared LOVD
We have used all reasonable efforts to ensure that the information displayed on these pages and contained in the databases is of high quality. We make no warranty, express or implied, as to its accuracy or that the information is fit for a particular purpose, and will not be held responsible for any consequences arising out of any inaccuracies or omissions. Individuals, organisations and companies which use this database do so on the understanding that no liability whatsoever either direct or indirect shall rest upon the curator(s) or any of their employees or agents for the effects of any product, process or method that may be produced or adopted by any part, notwithstanding that the formulation of such product, process or method may be based upon information here provided ...
https://databases.lovd.nl/shared/genes/GLA
Symptoms - Alpha-gal InformationSymptoms - Alpha-gal Information
All the information on alphagalinformation.org is provided in good faith, but we, the creators and authors of the Alpha-gal Information website offer no representation or warranty, explicit or implied, of the accuracy, adequacy, validity, reliability, availability, or completeness of any information on this site. Under no circumstances should we have any liability for any loss or damage incurred by you as a result of relying on information provided here. We are not physicians or medical professionals, researchers, or experts of any kind. Information provided in this website may contain errors and should be confirmed by a physician. Information provided here is not medical advice. It should not be relied upon for decisions about diagnosis, treatment, diet, food choice, nutrition, or any other health or medical decisions. For advice about health or medical decisions including, but not limited to, diagnosis, treatment, diet, and health care consult a physician ...
https://alphagalinformation.org/symptoms/
FAGPL - Specimen: Alpha-Gal PanelFAGPL - Specimen: Alpha-Gal Panel
FAGPL : Draw blood in a plain red-top tube(s), serum gel tube(s) is acceptable. Spin down and send 2 mL of serum refrigerated in a plastic vial.
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AGLA • Do Print | RisikorechnerAGLA • Do Print | Risikorechner
Berechnung des absoluten Risiko in %, innerhalb von 10 Jahren ein tödliches Koronaereignis (z.B. Herzinfarkt) oder einen nicht-tödlichen Herzinfarkt zu erleiden.. ...
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Systemic disease and the heart: Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective...
Background: Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. Objective: This study aimed to assess the long-term effects of ERT with recombinant α-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson-Fabry disease. Patients: Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson-Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). Results: At 45 months of treatment, LV mass and LV wall thickness had ...
http://connection.ebscohost.com/c/articles/43671861/systemic-disease-heart-effects-enzyme-replacement-therapy-patients-anderson-fabry-disease-prospective-long-term-cardiac-magnetic-resonance-imaging-study
Urinary podocyte loss is increased in patients with fabry disease and correlates with clinical severity of fabry nephropathy<...Urinary podocyte loss is increased in patients with fabry disease and correlates with clinical severity of fabry nephropathy<...
TY - JOUR. T1 - Urinary podocyte loss is increased in patients with fabry disease and correlates with clinical severity of fabry nephropathy. AU - Fall, Brent. AU - Scott, C. Ronald. AU - Mauer, Michael. AU - Shankland, Stuart. AU - Pippin, Jeffrey. AU - Jefferson, Jonathan A.. AU - Wallace, Eric. AU - Warnock, David. AU - Najafian, Behzad. PY - 2016/12. Y1 - 2016/12. N2 - Chronic kidney disease is a major complication of Fabry disease. Podocytes accumulate globotriaosylceramide inclusions more than other kidney cell types in Fabry patients. Podocyte injury occurs early in age, and is progressive. Since injured podocytes detach into the urine (podocyturia), we hypothesized that podocyturia would increase in Fabry patients and correlate with clinical severity of Fabry nephropathy. Urine specimens from 39 Fabry patients and 24 healthy subjects were evaluated for podocyturia. Most of the Fabry patients and many healthy subjects had podocyturia. The number of podocytes per gram of urine creatinine ...
https://experts.umn.edu/en/publications/urinary-podocyte-loss-is-increased-in-patients-with-fabry-disease
Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
Journal: EJNMMI - European Journal of Nuclear Medicine and Molecular Imaging ArticleTitle: Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
http://ejnmmigateway.net/ArticlePage.aspx?DOI=10.1007/s00259-017-3778-1
Fabry Disease 2011 - Bioinformatikpedia
Mutations of the gene GLA influence the enzyme α-galactosidase A. These mutations can affect the synthesis,ref name=lemansky,Lemansky et al., Synthesis and processing of alpha-galactosidase A in human fibroblasts. Evidence for different mutations in Fabry disease., J Biol Chem. 1987 Feb 15, PubMed,/ref,, kinetic properties and stability,ref name=bernstein,Bernstein et al., Fabry disease: six gene rearrangements and an exonic point mutation in the alpha-galactosidase gene., J Clin Invest 1989 Apr, PubMed,/ref, of the enzyme which leads to a decreased enzyme activity. Hence the catabolization of glycosphingolipids is not done properly which is especially the case for the breakdown of globotriaosylceramide (GL3) to lactosylceramide (GL2) and galactose (see Fig. 3 and 4),ref name=nance,Nance et al., Later-onset Fabry disease: an adult variant presenting with the cramp-fasciculation syndrome., Arch Neurol. 2006 Mar, PubMed,/ref,. Since the α-galactosidase A is located in the lysosome, Fabry ...
https://i12r-studfilesrv.informatik.tu-muenchen.de/wiki/index.php/Fabry_Disease_2011
Difference between revisions of Fabry Disease 2011 - Bioinformatikpedia
Mutations of the gene GLA influence the enzyme α-galactosidase A. These mutations can affect the synthesis,ref name=lemansky,Lemansky et al., Synthesis and processing of alpha-galactosidase A in human fibroblasts. Evidence for different mutations in Fabry disease., J Biol Chem. 1987 Feb 15, PubMed,/ref,, kinetic properties and stability,ref name=bernstein,Bernstein et al., Fabry disease: six gene rearrangements and an exonic point mutation in the alpha-galactosidase gene., J Clin Invest 1989 Apr, PubMed,/ref, of the enzyme which leads to a decreased enzyme activity. Hence the catabolization of glycosphingolipids is not done properly which is especially the case for the breakdown of globotriaosylceramide (GL3) to lactosylceramide (GL2) and galactose (see Fig. 3 and 4),ref name=nance,Nance et al., Later-onset Fabry disease: an adult variant presenting with the cramp-fasciculation syndrome., Arch Neurol. 2006 Mar, PubMed,/ref,. Since the α-galactosidase A is located in the lysosome, Fabry ...
https://i12r-studfilesrv.informatik.tu-muenchen.de/wiki/index.php?title=Fabry_Disease_2011&diff=16080&oldid=2028
A Study of the Safety and Efficacy of Fabrazyme in Patients With Fabry Disease - Full Text View - ClinicalTrials.govA Study of the Safety and Efficacy of Fabrazyme in Patients With Fabry Disease - Full Text View - ClinicalTrials.gov
People with Fabry Disease have an alteration in their genetic material (DNA) which causes a deficiency of the alpha-galactosidase A enzyme. Fabrazyme (agalsidase beta) is a drug that helps to break down and removes certain types of fatty substances called glycolipids. These glycolipids are normally present within the body in most cells. In Fabry disease, glycolipids build up in various tissues such as the liver, kidney, skin, and blood vessels because a-galactosidase A is not present, or is present in small quantities. The build up of glycolipid (globatriaosylceramide or GL-3) levels in these tissues in particular is thought to cause the clinical symptoms that are common to Fabry disease. This study analyzed the safety and efficacy of Fabrazyme in the treatment of patients with Fabry disease that previously participated in the AGAL-008-00 (NCT0074984) study ...
https://clinicaltrials.gov/show/NCT00081497
Central nervous system involvement in anderson-fabry diease: A clinical and MRI retrospective study | IRIS Università di PisaCentral nervous system involvement in anderson-fabry diease: A clinical and MRI retrospective study | IRIS Università di Pisa
BACKGROUND: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A. Central nervous system (CNS) manifestations consist mainly of cerebrovascular events. Brain MRI results are often abnormal. Purpose: The aim of the study was to describe CNS involvement in a group of Italian patients with AFD. METHODS: Clinical and brain MRI data of 43 patients with AFD (25 men, 41.94+/-10.83 years old and 18 women, 52.48+/-17.50 years old) were analysed retrospectively. 17 male patients and 7 female patients were under treatment with enzyme replacement therapy (ERT). RESULTS: All 43 patients had signs or symptoms of AFD. 16 men (64%) and 13 women (72%) demonstrated CNS involvement, although with varying severity. Overall, 6 men and 5 women had suffered from cerebrovascular accidents with an age at onset of 33.64+/-13.65 years and 53.68+/-11.71 years, respectively. Brain MR images were abnormal in 16/25 men and in 13/16 women. During CNS monitoring, ...
https://arpi.unipi.it/handle/11568/118610
Childrens Hospital of Iowa to test new treatment for Fabry disease - University News Service - The University of Iowa
Release: Dec. 4, 2002. Childrens Hospital of Iowa to test new treatment for Fabry disease. The Childrens Hospital of Iowa at University of Iowa Hospitals and Clinics will be one of 20 medical centers to take part in a major international study of a new treatment for Fabry disease, a rare genetic disorder that affects an estimated 1 in 40,000 males worldwide. The goal of the trial is to determine the safety and efficacy of recombinant human alpha-galactosidase A (Fabrazyme) on the progression of renal disease and significant clinical events in patients with Fabry disease. This will be a Phase IV multi-center, multinational, randomized, double-blind, placebo-controlled trial. Because Fabry disease is a rare genetic disorder, centers will need to work aggressively to identify patients to take part in this study. We will be working with families, Fabry patient organizations and medical centers throughout our area to identify patients for this very important clinical trial. We are very pleased to ...
http://www.news-releases.uiowa.edu/2002/december/1204fabry-disease.html
Fabry Disease Market Insights, Treatment, and Market Report by DelveInsight - Meerut ReporterFabry Disease Market Insights, Treatment, and Market Report by DelveInsight - Meerut Reporter
Fabry Disease Market: Report. The primary disease process starts in infancy, or even as early as in the fetal stage of development. In FD, lysosomal storage and cellular dysfunction are believed to trigger a cascade of events including cellular death, compromised energy metabolism, small vessel injury, K(Ca) channel dysfunction in endothelial cells, oxidative stress, impaired autophagosome maturation, tissue ischemia and, importantly, development of irreversible cardiac and renal tissue fibrosis.. Fabry Disease Market: Classification. Fabry Disease is classified as the Type 1 classic and Type 2 later-onset on the basis of phenotype of the disease. Both sub-types result in renal failure, and/or cardiac disease, and early death. Type 2 later-onset Fabry Disease is more frequent as compared to Type 1 classic Fabry Disease.. Fabry Disease Market: Symptoms. Symptoms of the disorders like Schindler disease, Gaucher disease, Fucosidosis, Erythromelalgia can be similar to those of Fabry disease. Thus ...
http://www.meerutreporter.com/story/114938/fabry-disease-market-insights-treatment-and-market-report-by-delveinsight.html
The Prevalence of Fabry Disease in Patients with Chronic Kidney Disease in Turkey: The TURKFAB Study | AVESİSThe Prevalence of Fabry Disease in Patients with Chronic Kidney Disease in Turkey: The TURKFAB Study | AVESİS
Background/Aims: Fabry disease is a treatable cause of chronic kidney disease (CKD) characterized by a genetic deficiency of a-galactosidase A. European Renal Best Practice (ERBP) recommends screening for Fabry disease in CKD patients. However, this is based on expert opinion and there are no reports of the prevalence of Fabry disease in stage 1-5 CKD. Hence, we investigated the prevalence of Fabry disease in CKD patients not receiving renal replacement therapy. Methods: This prospective study assessed a-galactosidase activity in dried blood spots in 313 stage 1-5 CKD patients, 167 males, between ages of 18-70 years whose etiology of CKD was unknown and were not receiving renal replacement therapy. The diagnosis was confirmed by GLA gene mutation analysis. Results: Three (all males) of 313 CKD patients (0.95%) were diagnosed of Fabry disease, for a prevalence in males of 1.80%. Family screening identified 8 aditional Fabry patients with CKD. Of a total of 11 Fabry patients, 7 were male and ...
https://avesis.ebyu.edu.tr/yayin/df732203-ee07-46b8-b196-66ac646b1c3b/the-prevalence-of-fabry-disease-in-patients-with-chronic-kidney-disease-in-turkey-the-turkfab-study
Control of proteinuria with increased doses of agalsidase alfa in a patient with Fabry disease with atypical genotype-phenotype...Control of proteinuria with increased doses of agalsidase alfa in a patient with Fabry disease with atypical genotype-phenotype...
Introduction. Fabry disease is a rare genetic lysosomal storage disorder of glycosphingolipids with X-linked transmission and an estimated incidence of 1:40,000-1:117,000 live male births.1 Partial or complete deficiency of the enzyme alpha-galactosidase A (a-Gal A) results in altered metabolism and progressive lysosomal accumulation of the substrate (mostly globotriaosylceramide, Gb3).2 The responsible gene is located on the long arm of the chromosome X (Xq22). More than 600 mutations have been identified with variable phenotypical expression.3. Clinically we distinguish the classical form and two variants, cardiac and renal. In the classical form clinical manifestations appear during childhood or early adolescence including acroparesthesias, angiokeratomas and corneal opacities.4 Progressive accumulation of Gb3 in the kidneys, heart and central nervous system lead to renal failure, hypertrophic cardiomyopathy and cerebral vascular accidents limiting life expectancy. The cardiac variant of the ...
https://revistanefrologia.com/es-control-proteinuria-with-increased-doses-articulo-S0211699515001332
OPUS Würzburg | SearchOPUS Würzburg | Search
Fabry disease is a rare X-linked hereditary disease caused by mutations in the AGAL gene encoding the lysosomal enzyme alpha-galactosidase A. Enzyme replacement therapy (ERT) is the current cornerstone of Fabry disease management. Involvement of kidney, heart and the central nervous system shortens life span, and fibrosis of these organs is a hallmark of the disease. Fibrosis was initially thought to result from tissue ischemia secondary to endothelial accumulation of glycosphingolipids in the microvasculature. However, despite ready clearance of endothelial deposits, ERT is less effective in patients who have already developed fibrosis. Several potential explanations of this clinical observation may impact on the future management of Fabry disease. Alternative molecular pathways linking glycosphingolipids and fibrosis may be operative; tissue injury may recruit secondary molecular mediators of fibrosis that are unresponsive to ERT, or fibrosis may represent irreversible tissue injury that ...
https://opus.bibliothek.uni-wuerzburg.de/solrsearch/index/search/searchtype/authorsearch/author/%22Politei%2C+Juan%22/start/0/rows/10/author_facetfq/Oritz%2C+Alberto
Ultrastructural deposits appearing as zebra bodies in renal biopsy: Fabry disease?- comparative case reports | BMC Nephrology...Ultrastructural deposits appearing as zebra bodies in renal biopsy: Fabry disease?- comparative case reports | BMC Nephrology...
In this paper, two cases of renal biopsy showing identical lipid deposits were presented. In the first case, these lipid deposits were due to inhibition of intralysosomal alpha-galactosidase A activity (presumable hydroxychloroquine-induced renal phospholipidosis) and in the second case, due to a mutation in the gene encoding the referred enzyme.. FD is a X-chromosome linked genetic disorder characterized by disturbance in glycosphingolipid catabolism, caused by a deficiency of the enzyme alpha-galactosidase A. Early signs and symptoms occur from childhood to adolescence and include intermittent paresthesia and acroparesthesia, Fabry crisis (episodes of intense pain), recurrent fever, angiokeratomas, cornea verticillata, mild proteinuria, globotriaosylceramide in urinary sediment and digestive symptoms such as both diarrhea and constipation, nausea, vomiting and abdominal cramps. Manifestations in adolescence and adulthood include renal disorders (which can progress to end-stage kidney ...
https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-017-0571-0
Fabrys disease and the kidney - Renal Fellow NetworkFabrys disease and the kidney - Renal Fellow Network
Fabrys is an X-linked lysosomal storage disorder caused by a deficiency in the enzyme alpha-galactosidase A. As a result, a buildup of glycosphyngolipids in cellular lysosomes occurs, a process thought to lead to cellular dysfunction. The effects are particularly seen in the nervous system, causing painful acroparesthesias; cardiac myocytes, leading to hypertrophic cardiomyopathy; and the kidneys, where endothelial storage of glycosphingolipids leads to vascular insufficiency and glomerular damage. A typical affected male will manifest frequent episodes of burning limb pain starting in childhood, which unfortunately respond poorly to analgesics. Other early signs of Fabrys include gastrointestinal pain and hypohydrosis, due to involvement of nerves innervating the gut and skin. Angiokeratomas, small reddish-purple lesions characteristic of the disease, are often present, especially in the groin area (and are often missed on physical examination).. Cardiac and renal involvement usually manifest ...
https://www.renalfellow.org/2010/05/06/fabrys-disease-and-kidney/
Fabry Disease - Rare Disease Quick Facts
Fabry disease is an X-linked lysosomal storage disorder that leads to excessive deposition of globotriaosylceramide ( GL-3) throughout the body. Skin, eye, kidney, heart, brain, and peripheral nervous system are highly vulnerable. Fabry disease is often difficult to diagnose since signs and symptoms are often nonspecific.. Symptoms. Many symptoms associated with Fabry disease are nonspecific making it a difficult disease to diagnosis. Not all symptoms may appear nor develop in any particular order. However, younger patients may have some or all of the following:. ...
http://www.raredr.com/news/fabry-disease-rare-disease-quick-facts
Discount China Wholesale alpha galactosidase 2000u/g,5kg/bag,free shipping [EP170508006]- US$406.00 - Allcosmeticsource.comDiscount China Wholesale alpha galactosidase 2000u/g,5kg/bag,free shipping [EP170508006]- US$406.00 - Allcosmeticsource.com
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PRIME PubMed | Agalsidase alfa in pediatric patients with Fabry disease: a 6.5-year open-label follow-up studyPRIME PubMed | Agalsidase alfa in pediatric patients with Fabry disease: a 6.5-year open-label follow-up study
PubMed journal article: Agalsidase alfa in pediatric patients with Fabry disease: a 6.5-year open-label follow-up study. Download Prime PubMed App to iPhone, iPad, or Android
https://www.unboundmedicine.com/medline/citation/25425121/Agalsidase_alfa_in_pediatric_patients_with_Fabry_disease:_a_6_5_year_open_label_follow_up_study_
Fabry disease: guidelines for the evaluation and management of multi-organ system involvementFabry disease: guidelines for the evaluation and management of multi-organ system involvement
Fabry disease is an X-linked metabolic storage disorder due to the deficiency of lysosomal alpha-galactosidase A, and the subsequent accumulation of glycosphingolipids, primarily globotriaosylceramide, throughout the body. Males with classical Fabry disease develop early symptoms including pain and …
https://pubmed.ncbi.nlm.nih.gov/16980809/?dopt=Abstract
Fabry diseaseFabry disease
Fabry disease is an X-linked dominant condition that affects 1 in 40,000 to 60,000 males. The prevalence in females is unknown. [1] Fabry disease results from mutations in the GLA gene encoding alpha-galactosidase A. The inability to metabolize glycosphingolipids causes a buildup in globotriaosylceramide in the cells found in nerves, vasculature, and other tissues including the cornea. This is a systemic disease that commonly presents as numbness and tingling of the hands and feet (acroparesthesias), skin lesions (angiokeratomas), decreased sweating (hypohidrosis), gastrointestinal dysfunction, kidney dysfunction, heart disease, vascular disease, and the following ophthalmic findings: corneal verticillata and vascular tortuosity. [2]. Corneal verticillata was first reported by Gruber in 1946 but it was not until 1969 when verticillata was attributed to Fabry disease by Franceschetti et al. [3,4] Corneal verticillata are also associated with many medications that cause subepithelial deposits and ...
https://www.eyerounds.org/cases/253-fabry-disease.htm
Nanobiotechnology: Human &-Galactosidase A Mutants: Priceless Tools to DevelopNovel Therapies for Fabry Disease - IBBNanobiotechnology: Human &-Galactosidase A Mutants: Priceless Tools to DevelopNovel Therapies for Fabry Disease - IBB
Abstract. Fabry disease (FD) is a lysosomal storage disease caused by mutations in the gene for the α-galactosidase A (GLA) enzyme. The absence of the enzyme or its activity results in the accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), in different tissues, leading to a wide range of clinical manifestations. More than 1000 natural variants have been described in the GLA gene, most of them affecting proper protein folding and enzymatic activity. Currently, FD is treated by enzyme replacement therapy (ERT) or pharmacological chaperone therapy (PCT). However, as both approaches show specific drawbacks, new strategies (such as new forms of ERT, organ/cell transplant, substrate reduction therapy, or gene therapy) are under extensive study. In this review, we summarize GLA mutants described so far and discuss their putative application for the development of novel drugs for the treatment of FD. Unfavorable mutants with lower activities and stabilities than wild-type enzymes ...
https://ibb.uab.cat/index.php/2021/06/23/nanobiotechnology-human-galactosidase-a-mutants-priceless-tools-to-developnovel-therapies-for-fabry-disease/
Childrens Hospital Of Iowa Research Benefits Fabry Patients - University News Service - The University of Iowa
Patients affected by a rare and potentially fatal inherited metabolic disorder called Fabry disease are now benefiting from a new treatment that researchers at Childrens Hospital of Iowa helped create.. Fabry disease affects approximately 5,000 people worldwide. The average lifespan of people diagnosed with the disorder is 50 years. The disease is caused by a deficiency of an enzyme called alpha-galactosidase A. Patients affected by the condition can develop kidney failure, strokes, heart disease and disabling pain. The Food and Drug Administration (FDA) recently approved a medication called Fabrazyme, produced by Genzyme General, for the treatment of Fabry disease. The therapy replaces the missing enzyme in order to help correct the disorder. The treatment took 30 years of research to develop.. Thomas Loew, M.D., associate professor (clinical) at Childrens Hospital of Iowa in University of Iowa Hospitals and Clinics, helped conduct the research that led to the FDAs approval of ...
http://news-releases.uiowa.edu/2003/august/082903fabry.html
Mosaicism of podocyte involvement is related to podocyte injury in females with Fabry diseaseMosaicism of podocyte involvement is related to podocyte injury in females with Fabry disease
Background: Fabry disease. an X-linked deficiency of α-galactosidase A coded by the GLA gene, leads to intracellular globotriaosylceramide (GL-3) accumulation. Although less common than in males, chronic kidney disease, occurs in ~15% of females. Recent studies highlight the importance of podocyte injury in Fabry nephropathy development and progression. We hypothesized that the greater the % of podocytes with active wild-type GLA gene (due to X-inactivation of the mutant copy) the less is the overall podocyte injury. Methods: Kidney biopsies from 12 treatment-naive females with Fabry disease, ages 15 (8-63), median [range], years were studied by electron microscopy and compared with 4 treatment-naive male patients.. Results: In females, 51 (13-100)% of podocytes (PC) per glomerulus had no GL-3 inclusions, this consistent with a non-Fabry podocyte phenotype (NFPC). In PC with GL-3 inclusions [Fabry podocyte phenotype (FPC)], GL-3 volume density per podocyte was virtually identical in females and ...
http://bora.uib.no/handle/1956/9699
Fabry Disease - OverviewFabry Disease - Overview
Enzyme replacement therapy can reduce storage, ease pain, and preserve organ function in some individuals with Fabry disease. Drugs are often prescribed to treat pain that accompanies Fabry disease but does not treat the disorder. The U.S. Food and Drug Administration has approved migalastat (Galafold) as an oral medication for adults with Fabry disease who have a certain genetic mutation. Anti-platelet medications can help prevent strokes and medications that lower blood pressure can slow the decline of kidney function in people with Fabry disease. ...
https://www.brainandlife.org/disorders/fabry-disease
Cardiac Microvascular Pathology in Fabry Disease | CirculationCardiac Microvascular Pathology in Fabry Disease | Circulation
Cardiac microvascular function abnormalities in Fabry patients have been demonstrated by measurements of myocardial blood flow and coronary flow reserve, an index of microvascular function.14 In addition, a survey of female Fabry patients revealed that cardiac ischemia could be confirmed by ECG and serological markers in the absence of coronary artery stenosis, suggesting that the ischemia in these patients was of microvascular origin.11 However, widespread coronary artery disease also has been documented in Fabry patients15 and noted at autopsy in a male patient who died of a massive myocardial infarction.16 In the present study, we characterized the baseline pathology of GL-3 accumulation in cardiac biopsies and demonstrated its successful clearance from the microvasculature after enzyme replacement therapy.. It has long been established that GL-3 is transported in low- and high-density lipoprotein particles17-19 and that vascular cells accumulate GL-3 from the circulation through the ...
http://circ.ahajournals.org/content/119/19/2561
Fabry disease - SNPediaFabry disease - SNPedia
Fabry disease is a rare enzyme deficiency known as a lysosomal storage disease. Wikipedia The enzyme involved, alpha galactosidase A, is coded by the GLA gene. OMIM Although Fabry disease has been considered an X-linked recessive condition, female carriers of a single mutated GLA gene may have significant symptoms. Enzyme replacement therapy is helpful, although it is currently extremely expensive, and production problems have led to shortages of the drug. [1] ...
https://snpedia.com/index.php/Fabry_disease
α-Galactosidase A - Sema4α-Galactosidase A - Sema4
Related Gene(s): GLA. Fabry disease is an X-linked lysosomal storage disorder caused by a marked deficiency of α-galactosidase A enzyme activity. Affected individuals are unable to degrade globotriaosylceramide (GL-3, also called Gb3) and related glycolipids in their lysosomes. The progressive accumulation of GL-3 and its derivative, lyso-GL-3, results in symptoms that include characteristic skin lesions (angiokeratomas), decreased sweating (hypohidrosis), chronic fatigue, depression, neuropathic pain in the hands and feet (acroparesthesia), gastrointestinal issues, strokes, cardiac disease (including left ventricular hypertrophy, leading to hypertrophic cardiomyopathy), and renal disease (proteinuria to end stage renal disease).. Fabry disease affects both men and women (heterozygotes), but the testing strategy varies based on sex. α-galactosidase A enzyme analysis alone detects all affected males, but is not reliable for the detection of Fabry disease in heterozygotes. Sequencing of the ...
https://sema4.com/products/test-catalog/alpha-galactosidase-a/
Department of Biochemistry and Molecular Biology | UMass AmherstDepartment of Biochemistry and Molecular Biology | UMass Amherst
Clark, NE, Garman, SC. The 1.9 A structure of human alpha-N-acetylgalactosaminidase: The structural basis of Schindler and Kanzaki diseases. Journal of Molecular Biology. 2009, Oct 23;393(2):435-447. [PubMed]. Ishii S, Chang HH, Kawasaki K, Yasuda K, Wu HL, Garman SC, Fan JQ. Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin. Biochem J. 2007 Sep 1;406(2):285-95.[PubMed]. Hebert DN, Garman SC, Molinari M. (2005) The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags. Trends Cell Biol. Jul;15(7):364-70. Review.[PubMed]. Su HP, Garman SC, Allison TJ, Fogg C, Moss B, Garboczi DN. (2005) The 1.51-Angstrom structure of the poxvirus L1 protein, a target of potent neutralizing antibodies. Proc Natl Acad Sci U S A. Mar 22;102(12):4240-5. Epub 2005 Mar 10. [PubMed]. Ries ...
http://www.umass.edu/biochem/faculty/scott-garman
High Incidence of Later-Onset Fabry Disease Revealed by Newborn Screening
In this article, alpha-galactosidase A activity was assayed in newborn screening blood spots of Italian male neonates. This study revealed a high incidence of later-onset Fabry disease.. For more information on Fabry disease, see chapter 150 of OMMBID ...
https://ommbidblog.com/2006/08/13/high-incidence-of-later-onset-fabry-disease-revealed-by-newborn-screening/
Third in a series on Fabry Disease: What is the importance of imaging in Fabry dThird in a series on Fabry Disease: What is the importance of imaging in Fabry d
Figure 1. Cardiac hypertrophy in a patient with Fabry disease. A. and B. Concentric LV hypertrophy. Note the presence of a hyperechogenic region in the posterior wall (mid-wall level, arrow), corresponding to localized fibrosis. C. Right ventricular free wall hypertrophy from subcostal view.. In most cases of FD, the LVH is concentric and non-obstructive; however, an asymmetrical hypertrophy with septal thickening and posterior wall fibrotic thinning may present in advanced cases. There have also been rare case reports of patients with obstructive forms of hypertrophic cardiomyopathy. The LVH is progressive in nature, and is rarely severe in children or adolescents. Of note, the echocardiographically derived cardiac mass is proportional to the electrocardiographic LVH low-voltage on the ECG, presenting an argument against Fabry disease in these patients.. Right ventricular (RV) hypertrophy is also common [9] (around 70% of Fabry patients display it) and may progress to RV dilation (Figure 1C). ...
https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-14/Third-in-a-series-on-Fabry-Disease-What-is-the-importance-of-imaging-in-Fabry-disease
Fabry disease in unselected patients with TIA or stroke: population-based study. - Radcliffe Department of MedicineFabry disease in unselected patients with TIA or stroke: population-based study. - Radcliffe Department of Medicine
BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder frequently associated with cerebrovascular disease. In recent years, the prevalence of FD has been reported to be up to 4% in cryptogenic young stroke patients. However, there have been no population-based studies in unselected patients with transient ischaemic attack (TIA) or stroke across the full range of ages. METHODS: We determined the prevalence of FD mutations in consecutive patients from a population-based study of acute TIA or ischaemic stroke (Oxford Vascular Study). Analysis included amplifying of the α-galactosidase A gene by polymerase chain reaction, denaturing high-performance liquid chromatography (dHPLC) analysis and sequencing using standard automated sequencing protocols [Mutation Surveyor software (Softgenetics)] where the dHPLC indicated a possible mutation. RESULTS: Samples of 1046 consecutive patients (52% women; mean age 73.2 years; 15% age |60 years; 572 stroke; 474 TIA) were tested. No patient had a known
https://www.rdm.ox.ac.uk/publications/325581
Shire Provides Update on Biologics License Application (BLA) Filing for REPLAGAL(R) (agalsidase alfa) With the U.S. Food and...Shire Provides Update on Biologics License Application (BLA) Filing for REPLAGAL(R) (agalsidase alfa) With the U.S. Food and...
Shire plc (LSE: SHP, NASDAQ: SHPGY), the global specialty biopharmaceutical company, announces it has received Fast Track designation from the FDA for REPLAGAL(R) (agalsidase alfa),
http://www.medindia.net/health-press-release/Shire-Provides-Update-on-Biologics-License-Application-BLA-Filing-for-REPLAGALR-agalsidase-alfa-With-the-US-Food-and-Drug--61855-1.htm
Alpha Galactosidase - Alpha Galactosidase Manufacturer,Supplier In Gujarat,IndiaAlpha Galactosidase - Alpha Galactosidase Manufacturer,Supplier In Gujarat,India
MITUSHI BIOPHARMA- Manufacturer & Supplier Of Alpha Galactosidase In Gujarat,India. Find More Detail For Alpha Galactosidase Manufacturers & Suppliers In Gujarat,India
https://www.mitushibiopharma.com/alpha-galactosidase-4111815.html
First oral treatment for Fabry disease recommended for approval in the EU - Pharma World
The European Medicines Agency (EMA) has recommended granting a marketing authorisation in the European Union (EU) for migalastat (Galafold) for the treatment of Fabry disease, a rare genetic disorder. Patients with Fabry disease do not have enough of an enzyme called alpha-galactosidase A.
http://www.pharmaworldmagazine.com/first-oral-treatment-for-fabry-disease-recommended-for-approval-in-the-eu/
Lack of susceptibility of cells from patients with Fabry disease to productive infection with R5 human immunodeficiency virus<...
TY - JOUR. T1 - Lack of susceptibility of cells from patients with Fabry disease to productive infection with R5 human immunodeficiency virus. AU - Lund, Nicole. AU - Branch, Donald R. AU - Sakac, Darinka. AU - Lingwood, Clifford A. AU - Siatskas, Christopher. AU - Robinson, Chevalia J. AU - Brady, Roscoe O. AU - Medin, Jeff. PY - 2005. Y1 - 2005. N2 - A lack of viral replication after HIV-1Ba-L (R5) but not HIV-1IIIB (X4) infection was found using in-vitro activated peripheral blood-derived mononuclear cells from patients with Fabry disease, who have a defect in the catabolism of globotriaosylceramide. CCR5, but not CD4 or CXCR4 expression levels, were lower and the surface expression of globotriaosylceramide was negligible on activated patients cells. Our findings suggest a novel resistance mechanism to productive infection with R5 HIV-1 that potentially involves abnormal globotriaosylceramide catabolism.. AB - A lack of viral replication after HIV-1Ba-L (R5) but not HIV-1IIIB (X4) infection ...
https://research.monash.edu/en/publications/lack-of-susceptibility-of-cells-from-patients-with-fabry-disease-
Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with (13)C...Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with (13)C...
We developed a mass spectrometric procedure to quantify sphingosine-1-phosphate (S1P) in biological materials. The use of newly synthesized (13)C5 C18-S1P and commercial C17-S1P as internal standards rendered very similar results with respect to linearity, limit of detection and limit of quantitation. Caution is warranted with determination of plasma S1P levels. Earlier it was reported that S1P is elevated in plasma of Fabry disease patients. We investigated this with the improved quantification. No clear conclusion could be drawn for patient plasma samples given the lack of uniformity of blood collection and plasma preparation. To still obtain insight, plasma and tissues were identically collected from α-galactosidase A deficient Fabry mice and matched control animals. No significant difference was observed in plasma S1P levels. A significant 2.3 fold increase was observed in kidney of Fabry mice, but not in liver and heart. Comparative analysis of S1P in cultured fibroblasts from normal ...
https://openaccess.leidenuniv.nl/handle/1887/43134
Fabry Disease Therapeutic Pipeline Review H1 2015: RnR Market ResearchFabry Disease Therapeutic Pipeline Review H1 2015: RnR Market Research
RnRMarketResearch.com offers Fabry Disease - Pipeline Review, H1 2015global research report on its store.. This report provides comprehensive information on the therapeutic development for Fabry Disease, complete with comparative analysis at various stages, therapeutics assessment by drug target, mechanism of action (MoA), route of administration (RoA) and molecule type, along with latest updates, and featured news and press releases. It also reviews key players involved in the therapeutic development for Fabry Disease and special features on late-stage and discontinued projects.. Global Markets Directs report features investigational drugs from across globe covering over 20 therapy areas and nearly 3,000 indications. The report is built using data and information sourced from Global Markets Directs proprietary databases, Company/University websites, SEC filings, investor presentations and featured press releases from company/university sites and industry-specific third party sources, put ...
https://pitchengine.com/pitches/578727d3-a567-4191-a2d5-172f3e628547
Fabrys Disease - CORE Kidney - Los Angeles, CAFabrys Disease - CORE Kidney - Los Angeles, CA
Fabrys disease is a multi-systemic disease, meaning it affects several systems of the body. Thus, Fabry management requires a multidisciplinary, expert team. The most common treatment is Enzyme Replacement Therapy, which aims to replace what the body is lacking. In this case, the body is lacking functional alpha-Gal A enzyme. The treatment is given in the form of bi-weekly infusions. Also, new oral medications (Chaperone therapy) can be administered for specific mutations. However, patients also require a comprehensive therapeutic approach from an expert team. The core team involved in the management of Fabrys disease usually includes a kidney doctor, a heart specialist, and a pain management specialist. Other doctors, including a digestive and lung disease specialist may be involved in the management as well.. Typically, physicians recommend the following:. ...
https://www.uclahealth.org/core-kidney/fabrys-disease-1075
Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal,...Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal,...
Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal, cardiac and stroke clinics, 1995-2017 ...
http://jmg.bmj.com/content/early/2018/01/12/jmedgenet-2017-105080.altmetrics
Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal,...Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal,...
Results Of 67 studies, 63 that screened 51363patients (33943M and 17420F) and provided GLA mutations were reanalysed for disease-causing mutations. Of reported GLA mutations, benign variants occurred in 47.9% of males and 74.1% of females. The following were the revised prevalence estimates: among 36820 (23954M and 12866F) haemodialysis screenees, 0.21% males and 0.15% females; among 3074 (2031M and 1043F) renal transplant screenees, 0.25% males and no females; among 5491 (4054M and 1437F) cardiac screenees, 0.94% males and 0.90% females; and among 5978 (3904M and 2074F) stroke screenees, 0.13% males and 0.14% females. Among male and female screenees with pathogenic mutations, the type 1 Classic phenotype was predominant (~60%), except more male cardiac patients (75%) had type 2 Later-Onset phenotype. ...
http://jmg.bmj.com/content/early/2018/01/12/jmedgenet-2017-105080.full
Alpha-galactosidaseAlpha-galactosidase
Dean KJ, Sweeley CC (October 1979). "Studies on human liver alpha-galactosidases. I. Purification of alpha-galactosidase A and ... alpha-galactosidase-A deficiency): physiopathology, clinical signs, and genetic aspects]" [Fabry's disease (α-galactosidase-A ... β-galactosidase Migalastat, a drug targeting α-galactosidase Classification of α-galactosidases (according to CAZy) GRCh38: ... alpha-Galactosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Human GLA genome location and GLA ...
https://en.wikipedia.org/wiki/Alpha-galactosidase
Capsular-polysaccharide endo-1,3-alpha-galactosidaseCapsular-polysaccharide endo-1,3-alpha-galactosidase
Capsular-polysaccharide+endo-1,3-alpha-galactosidase at the US National Library of Medicine Medical Subject Headings (MeSH) ... Capsular-polysaccharide endo-1,3-alpha-galactosidase (EC 3.2.1.87, polysaccharide depolymerase, capsular polysaccharide ... alpha-D-galactosidic linkages in Aerobacter aerogenes capsular polysaccharide Hydrolyses the galactosyl-alpha-1,3-D-galactose ...
https://en.wikipedia.org/wiki/Capsular-polysaccharide_endo-1,3-alpha-galactosidase
Blood group B branched chain alpha-1,3-galactosidaseBlood group B branched chain alpha-1,3-galactosidase
Heparanase (EC 3.2.1.166, Hpa1 heparanase, Hpa1, heparanase 1, heparanase-1, C1A heparanase, HPSE) is an enzyme with systematic name heparan sulfate N-sulfo-D-glucosamine endoglucanase. This enzyme catalyses the following chemical reaction endohydrolysis of (1->4)-beta-D-glycosidic bonds of heparan sulfate chains in heparan sulfate proteoglycan Heparanase cleaves the linkage between a glucuronic acid unit and an N-sulfo glucosamine unit carrying either a 3-O-sulfo or a 6-O-sulfo group. Heparanase Bame KJ (June 2001). "Heparanases: endoglycosidases that degrade heparan sulfate proteoglycans". Glycobiology. 11 (6): 91R-98R. doi:10.1093/glycob/11.6.91r. PMID 11445547. Peterson SB, Liu J (May 2010). "Unraveling the specificity of heparanase utilizing synthetic substrates". The Journal of Biological Chemistry. 285 (19): 14504-13. doi:10.1074/jbc.M110.104166. PMC 2863188. PMID 20181948. Pikas DS, Li JP, Vlodavsky I, Lindahl U (July 1998). "Substrate specificity of heparanases from human hepatoma and ...
https://en.wikipedia.org/wiki/Blood_group_B_branched_chain_alpha-1,3-galactosidase
Blood group B linear chain alpha-1,3-galactosidaseBlood group B linear chain alpha-1,3-galactosidase
Baicalin-beta-D-glucuronidase (EC 3.2.1.167, baicalinase) is an enzyme with systematic name 5,6,7-trihydroxyflavone-7-O-beta-D-glucupyranosiduronate glucuronosylhydrolase. This enzyme catalyses the following chemical reaction baicalin + H2O ⇌ {\displaystyle \rightleftharpoons } baicalein + D-glucuronate The enzyme also hydrolyses wogonin 7-O-beta-D-glucuronide and oroxylin 7-O-beta-D-glucuronide with lower efficiency. Ikegami F, Matsunae K, Hisamitsu M, Kurihara T, Yamamoto T, Murakoshi I (November 1995). "Purification and properties of a plant beta-D-glucuronidase form Scutellaria root". Biological & Pharmaceutical Bulletin. 18 (11): 1531-4. doi:10.1248/bpb.18.1531. PMID 8593473. Zhang C, Zhang Y, Chen J, Liang X (2005). "Purification and characterization of baicalin-β-D-glucuronidase hydrolyzing baicalin to baicalein from fresh roots of Scutellaria viscidula Bge". Proc. Biochem. 40: 1911-1915. doi:10.1016/j.procbio.2004.07.003. Sasaki K, Taura F, Shoyama Y, Morimoto S (September 2000). ...
https://en.wikipedia.org/wiki/Blood_group_B_linear_chain_alpha-1,3-galactosidase
GalactosidasesGalactosidases
If the galactoside is classified as an alpha-galactoside, the enzyme is called alpha-galactosidase, and is responsible for ... producing the alpha fragment and allowing for B-galactosidase to gain its activity. To trace the activity of B-galactosidase a ... Lack of alpha-galactosidase activity in leukocytes has been linked to Fabry Disease. Galactosidases have a variety of uses, ... the alpha-fragment gene would be inactive and the alpha fragment won't be produced. In that case B-galactosidase will not be ...
https://en.wikipedia.org/wiki/Galactosidases
Glycoside hydrolase family 4Glycoside hydrolase family 4
... alpha-galactosidase (EC 3.2.1.22); alpha-D-glucuronidase (EC 3.2.1.139). 6-phospho-alpha-glucosidase requires both NAD(H) and ... dependent 6-phospho-alpha-glucosidase. Assignment to family 4 of the glycosylhydrolase superfamily". The Journal of Biological ...
https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_4
Blue-white screenBlue-white screen
"Molecular basis of beta-galactosidase alpha-complementation". Proceedings of the National Academy of Sciences of the United ... β-galactosidase is a protein encoded by the lacZ gene of the lac operon, and it exists as a homotetramer in its active state. ... The presence of an active β-galactosidase can be detected by X-gal, a colourless analog of lactose that may be cleaved by β- ... However, a mutant β-galactosidase derived from the M15 strain of E. coli has its N-terminal residues 11-41 deleted and this ...
https://en.wikipedia.org/wiki/Blue–white_screen
Beano (dietary supplement)Beano (dietary supplement)
It contains the enzyme alpha-galactosidase (α-GAL). It was introduced as a liquid, but that has been discontinued and it is now ... Di Stefano M, Miceli E, Gotti S, Missanelli A, Mazzoccahi S, Corazza GR (January 2007). "The effect of oral alpha-galactosidase ... A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". J Fam Pract. 39 (5): ...
https://en.wikipedia.org/wiki/Beano_(dietary_supplement)
Glycoside hydrolase family 27Glycoside hydrolase family 27
Dey PM, Pridham JB (1972). "Biochemistry of α-Galactosidases". Biochemistry of alpha-galactosidases. Adv. Enzymol. Relat. Areas ... Alpha-galactosidase is present in a variety of organisms. There is a considerable degree of similarity in the sequence of alpha ... a superfamily of alpha-galactosidases, alpha-N-acetylgalactosaminidases, and isomaltodextranases which are likely to share a ... contains a region of about 50 amino acids which is similar to a domain of the eukaryotic alpha-galactosidases. Alpha-N- ...
https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_27
Glycoside hydrolase family 31Glycoside hydrolase family 31
... alpha-galactosidase (EC 3.2.1.22); glucoamylase (EC 3.2.1.3), sucrase-isomaltase (EC 3.2.1.48) (EC 3.2.1.10); alpha-xylosidase ... Hermans MM, Kroos MA, van Beeumen J, Oostra BA, Reuser AJ (July 1991). "Human lysosomal alpha-glucosidase. Characterization of ... Glycoside hydrolase family 31 CAZY GH_31 comprises enzymes with several known activities; alpha-glucosidase (EC 3.2.1.20), ... Homology with the rabbit intestinal sucrase-isomaltase complex and human lysosomal alpha-glucosidase". European Journal of ...
https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_31
Arthrobacter alkaliphilusArthrobacter alkaliphilus
It utilizes acid phosphatase, alkaline phosphatase, alpha-galactosidase, catalase, Pyrazinamidase, and urease. It is commonly ... alpha- and beta-glucuronidase, alpha-glucosidase, alpha-mannosidase, pyrazinamidase, pyrrolidonyl arylamidase, and urease. This ...
https://en.wikipedia.org/wiki/Arthrobacter_alkaliphilus
GlobosideGloboside
Germain, D. P. (2002). "Fabry's disease (alpha-galactosidase-A deficiency): Physiopathology, clinical signs, and genetic ... The side chain can be cleaved by galactosidases and glucosidases. The deficiency of α-galactosidase A causes Fabry's disease, ...
https://en.wikipedia.org/wiki/Globoside
GlobotriaosylceramideGlobotriaosylceramide
It is metabolized by alpha-galactosidase, which hydrolyzes the terminal alpha linkage. Defects in the enzyme alpha- ... The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabry's. ... a-Galactosidase A deficiency: Fabry disease. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic & molecular bases ... It is formed by the alpha linkage of galactose to lactosylceramide catalyzed by A4GALT. ...
https://en.wikipedia.org/wiki/Globotriaosylceramide
Penicillium ochrochloronPenicillium ochrochloron
Dey PM1, Patel S, Brownleader MD (June 1993). "Induction of alpha-galactosidase in Penicillium ochrochloron by guar (Cyamopsis ...
https://en.wikipedia.org/wiki/Penicillium_ochrochloron
FlatulenceFlatulence
A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". The Journal of Family ...
https://en.wikipedia.org/wiki/Flatulence
Robert J. DesnickRobert J. Desnick
National Institute of Diabetes and Digestive and Kidney Diseases Alpha Galactosidases A And B - Molecular and Cellular ... Cloning and expression of biologically active alpha-galactosidase A as a fusion protein, (1996). Acid sphingomyelinase gene and ... Human Development Cloning and expression of biologically active human alpha-galactosidase A, (1994). Cloning and expression of ... "Cloning and expression of biologically active human alpha-galactosidase A", published 1994-10-18, assigned to Mount Sinai ...
https://en.wikipedia.org/wiki/Robert_J._Desnick
Aspergillus nigerAspergillus niger
Alpha-galactosidase (GH27), an enzyme that breaks down certain complex sugars, is a component of Beano and several other ... Di Stefano M, Miceli E, Gotti S, Missanelli A, Mazzocchi S, Corazza GR (January 2007). "The effect of oral alpha-galactosidase ...
https://en.wikipedia.org/wiki/Aspergillus_niger
List of recombinant proteinsList of recombinant proteins
... pegfilgrastim sold as Neulasta alpha-galactosidase A: Fabrazyme by Genzyme alpha-L-iduronidase: (rhIDU; laronidase) Aldurazyme ...
https://en.wikipedia.org/wiki/List_of_recombinant_proteins
MigalastatMigalastat
The enzyme alpha-galactosidase A (α-GalA) Globotriaosylceramide (Gb3), a substrate of α-GalA, has a terminal D-galactose ... June 2009). "The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient ... of Fabry disease in adults and adolescents aged 16 or older with an amenable mutation of the enzyme alpha-galactosidase A (α- ... 2015). "Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients ...
https://en.wikipedia.org/wiki/Migalastat
Galactose-alpha-1,3-galactoseGalactose-alpha-1,3-galactose
A bacterial alpha-galactosidase that efficiently removes linear alpha-gal ends from molecules has been identified. It could be ... "Identification of a GH110 subfamily of alpha 1,3-galactosidases: novel enzymes for removal of the alpha 3Gal ... Galactose-alpha-1,3-galactose, commonly known as alpha gal and the Galili antigen, is a carbohydrate found in most mammalian ... Human reaction to alpha-gal has beneficial uses as a vaccine adjuvant and for enhancing wound healing. Alpha-gal allergy ...
https://en.wikipedia.org/wiki/Galactose-alpha-1,3-galactose
Streptomyces griseoloalbusStreptomyces griseoloalbus
Anisha, GS; Sukumaran, RK; Prema, P (March 2008). "Evaluation of alpha-galactosidase biosynthesis by Streptomyces griseoloalbus ... Anisha, G. S.; John, Rojan P.; Prema, P.; Pandey, Ashok (4 September 2008). "Investigation on α-Galactosidase Production by ... Anisha, G. S.; Prema, P. (5 December 2006). "Production of α-galactosidase by a novel actinomycete Streptomyces griseoloalbus ... "Production and characterization of partially purified thermostable α-galactosidases from Streptomyces griseoloalbus for food ...
https://en.wikipedia.org/wiki/Streptomyces_griseoloalbus
Glycoside hydrolase family 97Glycoside hydrolase family 97
The non-catalytic domains of glycosidases from the alpha-galactosidase and alpha-glucosidase superfamilies are also ... Naumoff DG (2005). "GH97 is a new family of glycoside hydrolases, which is related to the alpha-galactosidase superfamily". BMC ... In all known glycosidases with the (beta-alpha)8-barrel fold, the amino acid residues at the active site are located on the C- ... The central part of the GH97 family protein sequences represents a typical and complete (beta/alpha)8-barrel or catalytic TIM- ...
https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_97
Glycoside hydrolase family 57Glycoside hydrolase family 57
... α-galactosidase (EC 3.2.1.22); amylopullulanase (EC 3.2.1.41); branching enzyme (EC 2.4.1.18). It includes a thermostable alpha ... Glycoside hydrolase family 57 CAZY GH_57 comprises enzymes with several known activities; alpha-amylase (EC 3.2.1.1), 4-alpha- ... 1993). "The purification and characterization of an extremely thermostable alpha-amylase from the hyperthermophilic ...
https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_57
Fabry diseaseFabry disease
The lack of alpha-galactosidase leads to Fabry disease. A deficiency of alpha galactosidase A (a-GAL A, encoded by GLA) due to ... Fabry disease is an inherited lysosomal storage disorder that is caused by a deficiency of alpha-galactosidase A. This enzyme ... It does so by assisting correct folding of alpha-galactosidase despite the mutations that cause Fabry disease. As of March 2022 ... A person who inherits this gene does not have enough of a functioning enzyme known as alpha-galactosidase A. ...
https://en.wikipedia.org/wiki/Fabry_disease
CatarrhiniCatarrhini
... lost the enzyme Alpha-galactosidase, present in all other mammal lineages, sometime after the split from platyrrhini ... It is hypothesized that an ancient pathogen containing Alpha-galactosidase may be responsible, as only individuals with ... the gene for Alpha-galactosidase would have produced antibodies against the pathogen and survived. The distinction between apes ...
https://en.wikipedia.org/wiki/Catarrhini
Aspergillus sydowiiAspergillus sydowii
... contains an alpha-galactosidase enzyme; this enzyme, which hydrolyses the terminal alpha-galactosyl moieties from glycolipids ... Cai GL, Lu J (2012). "Isolation and identification of a novel Aspergillus sydowii F5 Producing alpha-galactosidase and ... is used in enzyme replacement therapy to functionally compensate for genetic alpha-galactosidase A deficiency. "Aspergillus ...
https://en.wikipedia.org/wiki/Aspergillus_sydowii
GalactosylgalactosylglucosylceramidaseGalactosylgalactosylglucosylceramidase
... ceramidetrihexoside alpha-galactosidase, trihexosylceramide alpha-galactosidase, and ceramidetrihexosidase. Brady RO, Gal AE, ... Other names in common use include trihexosyl ceramide galactosidase, ceramide trihexosidase, ...
https://en.wikipedia.org/wiki/Galactosylgalactosylglucosylceramidase
60S ribosomal protein L36a60S ribosomal protein L36a
1995). "Sixty-nine kilobases of contiguous human genomic sequence containing the alpha-galactosidase A and Bruton's tyrosine ...
https://en.wikipedia.org/wiki/60S_ribosomal_protein_L36a
Glycoside hydrolase family 36Glycoside hydrolase family 36
Alpha-galactosidase is present in a variety of organisms. There is a considerable degree of similarity in the sequence of alpha ... a superfamily of alpha-galactosidases, alpha-N-acetylgalactosaminidases, and isomaltodextranases which are likely to share a ... contains a region of about 50 amino acids which is similar to a domain of the eukaryotic alpha-galactosidases. Alpha-N- ... galactosidase from various eukaryotic species. Escherichia coli alpha-galactosidase (gene melA), which requires NAD and ...
https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_36
NAGA (gene)NAGA (gene)
1989). "Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B)". Biochem. ... Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene". J. Biol. Chem. 265 (35): 21859-66. ... Warner TG, Louie A, Potier M (1991). "Photolabeling of the alpha-neuraminidase/beta-galactosidase complex from human placenta ... 1996). "Human alpha-N-acetylgalactosaminidase (alpha-NAGA) deficiency: new mutations and the paradox between genotype and ...
https://en.wikipedia.org/wiki/NAGA_(gene)
Enzyme inhibitorEnzyme inhibitor
... alpha K_{m}+\alpha ^{\prime }[S]}}={\frac {(1/\alpha ^{\prime })V_{max}[S]}{(\alpha /\alpha ^{\prime })K_{m}+[S]}}} where the ... modeling beta-galactosidase and butyrylcholinesterase". Biochimica et Biophysica Acta (BBA) - General Subjects. 1770 (5): 733- ... alpha =1+{\frac {[I]}{K_{i}}}} α ′ = 1 + [ I ] K i ′ . {\displaystyle \alpha ^{\prime }=1+{\frac {[I]}{K_{i}^{\prime }}}.} Thus ... An example of a toxic peptide is alpha-amanitin, which is found in relatives of the death cap mushroom. This is a potent enzyme ...
https://en.wikipedia.org/wiki/Enzyme_inhibitor
Hypochlorous acidHypochlorous acid
The discovery that HClO blocks induction of β-galactosidase by added lactose led to a possible answer to this question. The ... The reaction of aqueous hypochlorous acid with alpha-amino acids and dipeptides". Biochimica et Biophysica Acta. 313 (1): 170- ... "Human neutrophils employ the myeloperoxidase-hydrogen peroxide-chloride system to oxidize alpha-amino acids to a family of ...
https://en.wikipedia.org/wiki/Hypochlorous_acid
P-selectinP-selectin
McEver RP, Beckstead JH, Moore KL, Marshall-Carlson L, Bainton DF (July 1989). "GMP-140, a platelet alpha-granule membrane ... Ligands for P-selectin on eosinophils and neutrophils are similar sialylated, protease-sensitive, endo-beta-galactosidase- ...
https://en.wikipedia.org/wiki/P-selectin
GcMAFGcMAF
List of ineffective cancer treatments Galactosidases - Advances in Research and Application. Scholarly Editions. 21 June 2013. ... misleadingly based on reduced levels of the alpha-N-acetylgalactosaminidase enzyme (also known as nagalase), whose production ...
https://en.wikipedia.org/wiki/GcMAF
Moss bioreactorMoss bioreactor
The enzyme alpha-galactosidase now is allowed to be produced in moss bioreactors by the German Federal Institute for Drugs and ...
https://en.wikipedia.org/wiki/Moss_bioreactor
Pseudogymnoascus destructansPseudogymnoascus destructans
... alpha/beta-galactosidase, β-glucuronidase, α-fucosidase, α-mannosidase, and trypsin. Important dual virulence factors found in ...
https://en.wikipedia.org/wiki/Pseudogymnoascus_destructans
Protalix BioTherapeuticsProtalix BioTherapeutics
... plant cell culture expressed and a chemically modified version of the recombinant alpha-Galactosidase-A protein. Protein sub- ...
https://en.wikipedia.org/wiki/Protalix_BioTherapeutics
Stem cell markerStem cell marker
August 2004). "Beta-galactosidase of ROSA26 mice is a useful marker for detecting the definitive erythropoiesis after stem cell ... Czerwinski M, Kiem HP, Slattery JT (March 1997). "Human CD34+ cells do not express glutathione S-transferases alpha". Gene ... October 2000). "The interleukin-3 receptor alpha chain is a unique marker for human acute myelogenous leukemia stem cells". ... asialo GM1 Bcl-2 Beta-galactosidase (β-gal) of ROSA26 mice beta1-integrin bromodeoxyuridine c-kit (CD117) c-Met C1qR(p) END ( ...
https://en.wikipedia.org/wiki/Stem_cell_marker
Beta-glucuronidaseBeta-glucuronidase
Human β-glucuronidase is homologous to the Escherichia coli enzyme β-galactosidase. This homologous relationship, along with ... Alpha-glucuronidase Glucuronosyl-disulfoglucosamine glucuronidase Glycyrrhizinate beta-glucuronidase PDB: 1BHG​; Jain S, ...
https://en.wikipedia.org/wiki/Beta-glucuronidase
GalactoseGalactose
A disaccharide composed of two units of galactose, galactose-alpha-1,3-galactose (alpha-gal), has been recognized as a ... The hydrolysis of lactose to glucose and galactose is catalyzed by the enzymes lactase and β-galactosidase. The latter is ... Alpha-gal allergy may be triggered by lone star tick bites. Galactose in sodium saccharin solution has also been found to cause ... In the cyclic form there are two anomers, named alpha and beta, since the transition from the open-chain form to the cyclic ...
https://en.wikipedia.org/wiki/Galactose
InulinaseInulinase
... which has one alpha helix. Being made of beta sheets allows the structures to be more stable during chemical reactions. Both of ... galactosidases, glucosidases and mannosidases". J. Am. Chem. Soc. 65 (7): 1369-1380. doi:10.1021/ja01247a029. Liu Y, Zhou SH, ...
https://en.wikipedia.org/wiki/Inulinase
Affinity chromatographyAffinity chromatography
Lectins, such as concanavalin A are proteins which can bind specific alpha-D-mannose and alpha-D-glucose carbohydrate molecules ... This property allows the enzyme to bind to the stationary phase of the affinity matrix and β-Galactosidase is eluted by adding ... For example, the purification of E. coli β-galactosidase is accomplished by affinity chromatography using p-aminobenyl-1-thio-β ...
https://en.wikipedia.org/wiki/Affinity_chromatography
SNCAIPSNCAIP
... stands for "synuclein, alpha interacting protein" and can be signified by SNCAP_HUMAN, synphilin 1, synuclein, alpha ... "Analysis of synphilin-1 and synuclein interactions by yeast two-hybrid beta-galactosidase liquid assay". Neurosci. Lett. 325 (2 ... Chung KK, Zhang Y, Lim KL, Tanaka Y, Huang H, Gao J, Ross CA, Dawson VL, Dawson TM (2001). "Parkin ubiquitinates the alpha- ... Tanaka M, Kim YM, Lee G, Junn E, Iwatsubo T, Mouradian MM (2004). "Aggresomes formed by alpha-synuclein and synphilin-1 are ...
https://en.wikipedia.org/wiki/SNCAIP
List of diseases (N)List of diseases (N)
... folate-sensitive Neural tube defects X linked Neuraminidase beta-galactosidase deficiency Neuraminidase deficiency Neurasthenia ... also Health Exercise Nutrition N acetyltransferase deficiency N syndrome N-Acetylglutamate synthase deficiency N-acetyl-alpha-D ...
https://en.wikipedia.org/wiki/List_of_diseases_(N)
List of enzymesList of enzymes
3-alpha,7-alpha-dihydroxy-5-beta-cholestanate-CoA ligase EC 6.2.1.29: Transferred entry: 6.2.1.7 EC 6.2.1.30: Phenylacetate-CoA ... Beta-galactosidase (EC 3.2.1.23) Hyaluronidase (EC 3.2.1.35) Function: Amylase is an enzyme that is responsible for the ...
https://en.wikipedia.org/wiki/List_of_enzymes
Snodgrassella alviSnodgrassella alvi
Tests of the strains for β-glucosidase, β-galactosidase, indole production, proteolysis of gelatin, and glucose fermentation ... phylum Actinobacteria), Gilliamella apicola, Frischella perrara, Bartonella apis, and Alpha 2.1 (phylum Proteobacteria). ...
https://en.wikipedia.org/wiki/Snodgrassella_alvi
Staphylococcus nepalensisStaphylococcus nepalensis
... beta-galactosidase, and beta-glucuronidase, and hydrolyses aesculin and Tween 80. It reduces nitrate to nitrite. It is negative ... alpha- and beta-haemolysins, heat-stable and heat-labile nucleases, indole, hydrogen sulphide, and lecithinase. It produces ...
https://en.wikipedia.org/wiki/Staphylococcus_nepalensis
TransferaseTransferase
"ABO ABO blood group (transferase A, alpha 1-3-N-acetylgalactosaminyltransferase; transferase B, alpha 1-3-galactosyltransferase ... Earliest discoveries of transferase activity occurred in other classifications of enzymes, including beta-galactosidase, ... "ABO Blood Group (Transferase A, Alpha 1-3-N-Acetylgalactosaminyltransferase;Transferase B, Alpha 1-3-Galactosyltransferase)". ... "ABO blood group (transferase A, alpha 1-3-N-acetylgalactosaminyltransferase; transferase B, alpha 1-3-galactosyltransferase)". ...
https://en.wikipedia.org/wiki/Transferase
Staphylococcus muscaeStaphylococcus muscae
... alpha- or beta-haemeolysins, ornithine decarboxylase, acetylmethylcarbinol, or beta-galactosidase. It does not hydrolyze starch ...
https://en.wikipedia.org/wiki/Staphylococcus_muscae
Beta-D-fucosidaseBeta-D-fucosidase
Chinchetru MA, Cabezas JA, Calvo P (1983). "Characterization and kinetics of beta-D-gluco/fuco/galactosidase from sheep liver ... beta-D-glucosides and alpha-L-arabinosides. ... "Beta-D-galactosidase and beta-D-fucosidase of pig kidney". ... Rodriguez JA, Cabezas JA, Calvo P (1982). "beta-Fucosidase, beta-glucosidase and beta-galactosidase activities associated in ...
https://en.wikipedia.org/wiki/Beta-D-fucosidase
AntiflatulentAntiflatulent
... it may be helpful to take a product that contains alpha-galactosidase, such as Beano[citation needed] or kombu. Additionally, ...
https://en.wikipedia.org/wiki/Antiflatulent
GLAGLA
... a fictional comic hero group Alpha-galactosidase, a glycoside hydrolase enzyme GLA (gene), an encoding of the enzyme alpha- ... galactosidase A Gla domain, a protein domain Gamma-Linolenic acid, a fatty acid Linde-Buzo-Gray algorithm (also called ...
https://en.wikipedia.org/wiki/GLA
Enzyme kineticsEnzyme kinetics
December 1966). "The determination of the concentration of hydrolytic enzyme solutions: alpha-chymotrypsin, trypsin, papain, ... modeling beta-galactosidase and butyrylcholinesterase". Biochimica et Biophysica Acta (BBA) - General Subjects. 1770 (5): 733- ...
https://en.wikipedia.org/wiki/Enzyme_kinetics
Dose-Ranging Study of ST-920, an AAV2/6 Human Alpha Galactosidase A Gene Therapy in Subjects With Fabry Disease - Full Text...Dose-Ranging Study of ST-920, an AAV2/6 Human Alpha Galactosidase A Gene Therapy in Subjects With Fabry Disease - Full Text...
Dose-Ranging Study of ST-920, an AAV2/6 Human Alpha Galactosidase A Gene Therapy in Subjects With Fabry Disease. The safety and ... an AAV2/6 Human Alpha Galactosidase A Gene Therapy, in Subjects With Fabry Disease. ...
https://clinicaltrials.gov/ct2/show/NCT04046224
Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) | CSB-YP356371SAC | CusabioRecombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) | CSB-YP356371SAC | Cusabio
Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) from Cusabio. Cat Number: CSB-YP356371SAC. USA, UK & Europe ... Recombinant Human Alpha-galactosidase A (GLA) , CSB-EP009474HU , CusabioAlternative Name(s): Alpha-D-galactosidase AAlpha-D- ... Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) , CSB-YP356371SAC. (No reviews yet) Write a Review Write a ... Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) , CSB-YP356371SAC , Cusabio. Alternative Name(s): Alpha-D- ...
https://joplink.net/recombinant-saccharomyces-cerevisiae-alpha-galactosidase-1-mel1-csb-yp356371sac/
Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype |...Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype |...
... caused by mutations of the alpha-galactosidase A (GLA) gene. We analyzed 15 patients carrying the GLA haplotype -10C>T [ ... A deficiency of alpha-galactosidase A (GLA, E.C.3.2.1.22) leads to Fabry disease (FD), an X-linked lysosomal storage disorder. ... Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype. *Michael ... Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype ...
https://ojrd.biomedcentral.com/articles/10.1186/s13023-014-0178-5
PDF] The Effect of Oral α-Galactosidase on Intestinal Gas Production and Gas-Related Symptoms | Semantic ScholarPDF] The Effect of Oral α-Galactosidase on Intestinal Gas Production and Gas-Related Symptoms | Semantic Scholar
α-Galactosidase reduced gas production following a meal rich in fermentable carbohydrates and may be helpful in patients with ... Eight healthy volunteers ingested 300 or 1200 GalU of α-galactosidase or placebo during a test meal containing 420 g of cooked ... The aim was to evaluate the effect of α-galactosidase administration, in a randomized double-blind placebo-controlled protocol ... The administration of 1200 GalU of α-galactosidase induced a significant reduction of both breath hydrogen excretion and ...
https://www.semanticscholar.org/paper/The-Effect-of-Oral-%CE%B1-Galactosidase-on-Intestinal-Stefano-Miceli/214f050f68904a6552710e109ca2f3f76bdc5b14
Alpha-galactosidase - Intentional WellnessAlpha-galactosidase - Intentional Wellness
Alpha-galactosidase. The Challenges of a Raw Food Diet. Raw Food Diet - Good or Bad? You often see things on Pinterest and ...
https://intentionalwellnessinc.com/tag/alpha-galactosidase
IMSEAR at SEARO: Immobilization of alpha-galactosidase from coconut endosperm
Alpha-galactosidase was covalently bound to nylon pellets with a percentage binding of 14 percent and a retained activity of 49 ... When Alpha-galactosidase was covalently bound to nylon powder there was a percentage binding of 19 percent and a retained ... Alpha-galactosidase was also immobilized on Sepharose-4B-caproic acid with a percentage binding of 48 percent and a retained ... Purified Alpha-galactosidase (60 units) was immobilized on Sepharose-4B-capranilide gel (72 ml) in the presence of ammonium ...
https://imsear.searo.who.int/handle/123456789/129479
A-Z Drug Index for Prescription and OTC MedicationsA-Z Drug Index for Prescription and OTC Medications
Alpha galactosidase Alpha-d-galactosidase is an enzyme used to digest complex carbohydrates in the food and helps in digestion. ... Alpha One-proteinase inhibitor Alpha One-proteinase inhibitor is prescribed to treat alpha -1 antitrypsin deficiency, a genetic ... Alpha-linolenic acid Alpha-linolenic acid is used to prevent or treat the disease of heart and blood vessels by reducing the ... a rare genetic disorder occurs due to a deficiency of alpha-galactosidase-A (an enzyme responsible for breaking down fat). ...
https://www.medindia.net/doctors/drug_information/home.asp
MedlinePlus: Genes: GMedlinePlus: Genes: G
GLA: galactosidase alpha. *GLB1: galactosidase beta 1. *GLDC: glycine decarboxylase. *GLI3: GLI family zinc finger 3 ... GNPTAB: N-acetylglucosamine-1-phosphate transferase subunits alpha and beta. *GNPTG: N-acetylglucosamine-1-phosphate ...
https://medlineplus.gov/genetics/gene-g/
Effects of Alpha Galactosidase Enzyme on Feed Products - BESTZYME BIO-ENGINEERING CO., LTD.Effects of Alpha Galactosidase Enzyme on Feed Products - BESTZYME BIO-ENGINEERING CO., LTD.
Alpha-galactosides are composed of a sucrose unit and one (or more) galactose molecules connected by α1,6 glycosidic bonds. ... Products Phytase Lipase Glucose Oxidase Beta Mannanase Xylanase Beta Glucanase Cellulase Alpha Galactosidase Pectinase Alpha ... Alpha galactosidase enzyme is the enzyme containing alpha galactosides bonds after the catalytic hydrolysis process. It can ... Alpha galactosidase enzyme is an endonuclease derived from fungal liquid submerged fermentation. The average particle size is ...
https://www.bestzyme.com/effects-of-alpha-galactosidase-enzyme-on-feed-products.html
NAGA alpha-N-acetylgalactosaminidase [Homo sapiens (human)] - Gene - NCBINAGA alpha-N-acetylgalactosaminidase [Homo sapiens (human)] - Gene - NCBI
Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B). N-acetylgalactosaminidase, alpha-. alpha-galactosidase B. NP_ ... the active sites of human lysosomal enzymes alpha-galactosidase and alpha-N-acetylgalactosaminidase have interconvertible ... Three dimensional structural studies of alpha-N-acetylgalactosaminidase (alpha-NAGA) in alpha-NAGA deficiency (Kanzaki disease ... enables alpha-galactosidase activity IBA Inferred from Biological aspect of Ancestor. more info ...
https://www.ncbi.nlm.nih.gov/gene/4668
Fabry Disease: Symptoms, Treatment and Life ExpectancyFabry Disease: Symptoms, Treatment and Life Expectancy
Fabry disease is a rare genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a ... Fabry disease is a rare genetic disease with a deficiency of an enzyme called alpha-galactosidase A. The disease affects many ... Fabry disease is caused by a genetic mutation resulting in a deficiency of an enzyme called alpha-galactosidase A (a-Gal A). ... Fabry disease is the result of a mutation of the gene responsible for making alpha-galactosidase enzyme, which is located on ...
https://www.medicinenet.com/fabrys_disease/article.htm
Digestive Enzymes and Weight Loss: Do They Help?Digestive Enzymes and Weight Loss: Do They Help?
Alpha-galactosidase: helps break down complex carbs in beans, vegetables, and grains ...
https://www.healthline.com/nutrition/digestive-enzymes-and-weight-loss
Fabrys disease presenting with strokeFabry's disease presenting with stroke
Rainbow Light Active Adult 50+ on sale at AllStarHealth.comRainbow Light Active Adult 50+ on sale at AllStarHealth.com
cellulase (24 CU†), bromelain (5102 PU†), alpha-galactosidase (4 GalU†), lipase (23 FIP†), lactase. ...
https://www.allstarhealth.com/en-gb/f/rainbow_light-just_once_active_one_senior_multi.htm
Human Genome Variation Society
galactosidase, alpha 300644 GLA database at LOVD Johan T. den Dunnen Leiden Univ. Med Centre (acting), Curator vacancy ... galactosidase, alpha 300644 CCHMC - Human Genetics Mutation Database Ammar Husami and Theru A. Sivakumaran ... glycine receptor, alpha 4 \N GLRA4 database at LOVD Johan T. den Dunnen Leiden Univ. Med Centre (acting), Curator vacancy ... glycine receptor, alpha 2 305990 GLRA2 database at LOVD Johan T. den Dunnen Leiden Univ. Med Centre (acting), Curator vacancy ...
http://www.hgvs.org/locus-specific-mutation-databases/g?field_hgnc_gene_symbol_title=
Publications | Nichols LabPublications | Nichols Lab
Alpha galactosidase A activity in Parkinsons disease. Neurobiology of Disease. 2018; 112:85-90. ...
https://www.cincinnatichildrens.org/research/divisions/h/genetics/labs/nichols/publications
Lysosomal Storage Disease: Overview, Classification of Lysosomal Storage Diseases, Glycogen Storage Disease Type IILysosomal Storage Disease: Overview, Classification of Lysosomal Storage Diseases, Glycogen Storage Disease Type II
Schindler disease results from the deficient activity of the enzyme alpha-N -acetylgalactosaminidase (alpha-galactosidase B), ... Novel sequence variants of the alpha-galactosidase A gene in patients with Fabry disease. Mol Genet Metab. 2008 Dec. 4:224-8. [ ... Evaluation of recombinant alpha-galactosidase A therapy for amelioration of the cardiovascularmanifestations of Fabry disease: ... Fabry disease (alpha-galactosidase A) (Also see the Medscape Reference article Fabry Disease.) ...
https://emedicine.medscape.com/article/1182830-overview
Yanhong Liu : USDA ARSYanhong Liu : USDA ARS
Galacto-oligosaccharide hydrolysis by genetically-engineered alpha-galactosidase-producing Pseudomonas chlororaphis strains - ( ... Galacto-oligosaccharide hydrolysis by genetically-engineered alpha-galactosidase-producing Pseudomonas chlororaphis strains. ...
https://www.ars.usda.gov/people-locations/person?person-id=35470
Osteocalcin Antibody (190125) (MAB1419): Novus BiologicalsOsteocalcin Antibody (190125) (MAB1419): Novus Biologicals
Cited in 31 publications. View Mouse Monoclonal anti-Osteocalcin Antibody (190125) (MAB1419). Validated Applications: IHC, CyTOF-ready, Flow, ICC/IF. Validated Species: Human, Rat. Sample size available. Range of directly conjugated antibodies available.
https://www.novusbio.com/products/osteocalcin-antibody-190125_mab1419
Omega-Zyme Omega Zyme 180 Caplets Garden of Life - Compare Price and Reviews at VitaDigest.comOmega-Zyme Omega Zyme 180 Caplets Garden of Life - Compare Price and Reviews at VitaDigest.com
alpha-Galactosidase. 475 GaLU. +. Cellulase. 663 CU. +. Pectinase. 15.5 endo-PG. +. Xylanase. 550 XU. +. ...
https://www.vitadigest.com/gl-o-zyme-180.html
Fabry disease: Symptoms, causes, and treatmentFabry disease: Symptoms, causes, and treatment
Fabry disease results when there is a shortage of an enzyme called alpha-galactosidase A (a-GAL A). This enzyme is is encoded ... In Fabry disease patients cases, the missing enzyme is alpha-galactosidase A (a-GAL A). ... Other names include Anderson-Fabry disease, alpha-galactosidase A deficiency, or angiokeratoma corporis diffusum. ... What is 5 alpha reductase deficiency? Learn about this genetic condition, including treatment options and how it differs from ...
https://www.medicalnewstoday.com/articles/263333?utm_source=ReadNext
Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry | Journal...Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry | Journal...
Alpha-galactosidase A deficiency: Fabry disease. In: Scriver CR, Beaudet AL, Valle D, Sly WS, Childs B, Kinzler KW, Vogelstein ... Detection of 8 new mutations in the alpha-galactosidase A gene in Fabry disease. Hum Mol Genet 1994;3:667-9. doi:10.1093/hmg/ ... The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and ... Safety and efficacy of recombinant human alpha-galactosidase A--replacement therapy in Fabrys disease. N Engl J Med 2001;345:9 ...
https://jmg.bmj.com/content/53/7/495
Giddyap Girls BioEZ - KVSupply.comGiddyap Girls BioEZ - KVSupply.com
Alpha Galactosidase: 280 GalU. Lactic Acid Producting Bacteria**: 400 million CFU* **Lactobacillus Acidophilus, Bifidobacterium ... Alpha Amylase: 1100 DU. Acid beta Cellulase: 2250 CU. Neutral beta Cellulase: 200 CU. Beta Glucanase: 1100 beta GU. Acid ...
https://www.kvsupply.com/item/giddyap-girls-bioez/e016499/
Journal of Zhejiang University SCIENCE BJournal of Zhejiang University SCIENCE B
alpha-galactosidase induced by the constitutive PGK1 promoter was used as a food-grade selection marker. The feasibility of the ... and tumor necrosis factor-alpha (TNF-alpha). Based on these results, the anti-inflammatory effects and low cell toxicity of ... alpha-galactosidase marker was confirmed by the growth of transformants harboring the constructed vector on a medium containing ... pH had little effect on beta-glucanase or proteases partition but affected alpha-amylase partition when pH was over 7.0. The ...
https://www.researchgate.net/journal/Journal-of-Zhejiang-University-SCIENCE-B-1862-1783
Solaray Once Daily Adult 50+ on sale at AllStarHealth.comSolaray Once Daily Adult 50+ on sale at AllStarHealth.com
B-Glucanase, B-Glucosidase, Pectinase, Alpha-Galactosidase. Herb & Food Base. 126mg. †. -Eleuthero (root), Astragalus (root), ... Vitamin E (d-Alpha Tocopheryl Succinate) (Supplying 60 IU of Vitamin E). 40mg. 267. ...
https://www.allstarhealth.com/en-gb/de_p/47679/Solaray_Once_Daily_Adult_50PLUS.htm
UVigoTV - 3rd Annual Meeting CinbioUVigoTV - 3rd Annual Meeting Cinbio
Functional evaluation of an AAV9 vector expressing alpha-Galactosidase A for potential gene therapy of Fabry disease. 1 de xul ... Functional evaluation of an AAV9 vector expressing alpha-Galactosidase A for potential gene therapy of Fabry disease. 1 de xul ...
https://tv.uvigo.es/series/5d6fa4a01e19c03d952b8c89
Master Supplements Enzalase - Nutrition Depot OnlineMaster Supplements Enzalase - Nutrition Depot Online
Alpha-Amylase 5,000 SKB. * Alpha-Galactosidase 165 GALU. * Bromelain Protease 200 GDU ...
https://ndonline.com/products/master-supplements-enzalase
BeanAssist 30 capsules - BayhoBeanAssist 30 capsules - Bayho
Serving Size: 1 Capsule Alpha Galactosidase ... 300GaIU Other Ingredients: 100% vegetarian capsule (cellulose, water) Does not ... Alpha Galactosidase ... 300GaIU. Other Ingredients: 100% vegetarian capsule (cellulose, water). Does not contain egg, dairy, ...
https://bayho.com/products/beanassist-30-capsules
Garden of Life OmegaZyme Ultra Digestive Enzymes 180 capsGarden of Life OmegaZyme Ultra Digestive Enzymes 180 caps
Alpha-Galactosidase. 450 GalU. *. Amylase. 25000 DU. *. Fiber Digestive Blend. (Phytase, Hemicellulase, beta-Glucanase). 20 mg ...
https://www.crohns.net/catalog/product/view/id/165/s/garden-of-life-omegazyme-ultra-digestive-enzymes-180-caps/
KEGG REACTION: R12962KEGG REACTION: R12962
alpha-galactosidase [EC:3.2.1.22]. K07407 alpha-galactosidase [EC:3.2.1.22]. K07553 beta-1,4-galactosyltransferase 6 [EC:2.4. ... aklavinone 7-alpha-L-rhodosaminyltransferase [EC:2.4.1.326]. K15947 aklavinone 7-alpha-L-rhodosaminyltransferase auxiliary ... 1,2-diacylglycerol-3-alpha-glucose alpha-1,2-glucosyltransferase [EC:2.4.1.208]. ... phosphatidyl-myo-inositol alpha-mannosyltransferase [EC:2.4.1.345]. K09654 beta-1,3-N-acetylgalactosaminyltransferase 2 [EC:2.4 ...
https://www.kegg.jp/entry/R12962+RC00049
DeficiencyAccumulation of glycosphingolipidsFabryRaffinoseEnzymaticHydrolaseSoybeanLysosomalHydrolysisEnzyme activityActivityPelletsProteinStructuralBetaDietLevelsHighActiveTreatmentAmylaseLipaseLactaseInvertaseBeta-glucanaseEnzymes1.22GeneLactoseDeficiency of alpha-galactosidaseBlendFabry DiseaseProteinBeanoEscherichiaTrichodermaCatalyticLipoic AcidAmenableStarchesDigestionEnzyme replaceDeficientCarbohydratesAspergillusGalactoseGlobotriaosylceramideSaccharomycesCharacterizationLack the enzymeSubstrateActive ingredientBreakdownBacteriaUnitsExtractGalactomannans
Deficiency10
  • A deficiency of alpha-galactosidase A (GLA, E.C.3.2.1.22) leads to Fabry disease (FD), an X-linked lysosomal storage disorder. (biomedcentral.com)
  • A new infantile case of alpha-N-acetylgalactosaminidase deficiency. (nih.gov)
  • Three dimensional structural studies of alpha-N-acetylgalactosaminidase (alpha-NAGA) in alpha-NAGA deficiency (Kanzaki disease): different gene mutations cause peculiar structural changes in alpha-NAGAs resulting in different substrate specificities and clinical phenotypes. (nih.gov)
  • Fabry disease is a rare genetic disease with a deficiency of an enzyme called alpha-galactosidase A. The disease affects many parts of the body including the skin, eyes, gastrointestinal system, kidney, heart , brain, and nervous system. (medicinenet.com)
  • Fabry disease is a rare genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a type of fat called globotriaosylceramide (Gb3, or GL-3) in the body. (medicinenet.com)
  • Fabry disease is caused by a genetic mutation resulting in a deficiency of an enzyme called alpha-galactosidase A (a-Gal A). The genetic defect causes the body to not make enough of this enzyme leading to a buildup of a type of fat called globotriaosylceramide (Gb3, or GL-3) in the body cause the symptoms of Fabry disease. (medicinenet.com)
  • Other names include Anderson-Fabry disease, alpha-galactosidase A deficiency, or angiokeratoma corporis diffusum. (medicalnewstoday.com)
  • Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (⍺-GalA). (biomedcentral.com)
  • Dietary restriction has shown promise for disorders such as lysosomal acid lipase deficiency (Wolman disease), as has incorporation of lipid-lowering drugs in the regimen along with sebelipase alpha, a recombinant enzyme replacement therapy. (medscape.com)
  • BACKGROUND AND OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal storage disease with various clinical symptoms due to a deficiency of an enzyme called alpha-galactosidase A. The likelihood of nephropathy increases with age and the severity of the mutation in Fabry patients. (bvsalud.org)
Accumulation of glycosphingolipids2
  • Background Agalsidase β is a form of enzyme replacement therapy for Fabry disease, a genetic disorder characterised by low α-galactosidase A activity, accumulation of glycosphingolipids and life-threatening cardiovascular, renal and cerebrovascular events. (bmj.com)
  • Fabry disease is an X-linked lysosomal storage disorder caused by reduced activity or absence of the alpha-galactosidase A enzyme resulting in systemic accumulation of glycosphingolipids. (bmj.com)
Fabry3
  • Fabry disease (FD) is a multisystemic disorder with typical neurological manifestations such as stroke and small fiber neuropathy (SFN), caused by mutations of the alpha-galactosidase A ( GLA ) gene. (biomedcentral.com)
  • Fabry disease is the result of a mutation of the gene responsible for making alpha-galactosidase enzyme, which is located on the X chromosome. (medicinenet.com)
  • Fabry disease results when there is a shortage of an enzyme called alpha-galactosidase A (a-GAL A). This enzyme is is encoded by the GLA gene. (medicalnewstoday.com)
Raffinose2
  • The purified enzyme was shown to be homogeneous by polyacrylamide gel electrophoresis and isolectric focusing.Studies on inhibition of Alpha-galactosidase by carbohydrates showed that the enzyme is no inhibited by sucrose and glucose and only 29 percent inhibited by a 1 percent raffinose solution. (who.int)
  • We know of one enzyme, alpha-galactosidase, that can break down raffinose, but it isn't present in our stomachs. (zmescience.com)
Enzymatic1
  • Three additional (3.4%) patients had the enzymatic activity of alpha-galactosidase slightly decreased. (biomedcentral.com)
Hydrolase1
  • Alpha/beta hydrolase family [Interproscan]. (ntu.edu.sg)
Soybean1
  • 6. Application scope of alpha galactosidase enzyme: soybean meal or miscellaneous meal-type pellets, concentrates and premixes of pigs, poultry and aquatic products. (bestzyme.com)
Lysosomal1
  • NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. (nih.gov)
Hydrolysis3
  • Alpha galactosidase enzyme is the enzyme containing alpha galactosides bonds after the catalytic hydrolysis process. (bestzyme.com)
  • An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids. (bvsalud.org)
  • Both saposin B and C have in vitro effects on hydrolysis of LacCer by activation of galactosylceramide-β-galactosidase, GM1-β-galactosidase and other β-galactosidases ( 10 ). (cdc.gov)
Enzyme activity1
  • The disease is diagnosed with a test that measures leukocyte alpha-Gal A enzyme activity. (medicinenet.com)
Activity9
  • Purified Alpha-galactosidase (60 units) was immobilized on Sepharose-4B-capranilide gel (72 ml) in the presence of ammonium sulphate with 100 percent binding and 59 percent retained activity. (who.int)
  • When 7.75 units of Alpha-galactosidase was immobilized by entrapment in the K-carrageenan gel (20 ml) there was a percentage binding of 57 percent and a retained activity of 54 percent. (who.int)
  • Alpha-galactosidase was covalently bound to nylon pellets with a percentage binding of 14 percent and a retained activity of 49 percent. (who.int)
  • When Alpha-galactosidase was covalently bound to nylon powder there was a percentage binding of 19 percent and a retained activity of 64 percent. (who.int)
  • Alpha-galactosidasewas covalently bound to Sepharose-4B-lysine with a percentage binding of 36 percent and a retained activity of 2.25 percent. (who.int)
  • Alpha-galactosidase was also immobilized on Sepharose-4B-caproic acid with a percentage binding of 48 percent and a retained activity of 32 percent. (who.int)
  • At 37°C and pH 5.5, the amount of enzyme required to degrade and release 1 μmol of p-nitrophenol from a 10mM concentration of p-nitrophenol αD galactopyranoside solution per minute is an α-galactosidase activity Unit (IU). (bestzyme.com)
  • The five longest treated patients continued to exhibit elevated alpha-galactosidase A (α-Gal A) activity, sustained up to 15 months as of the last date of measurement. (businesswire.com)
  • Alpha galactosidase A activity in Parkinson's disease. (cdc.gov)
Pellets1
  • Alpha-galactosidase immobilized in the 15 percent polyacrylamide gel, K-carrageenan gel, nylon pellets and nylon power were used to study the properties of the immobilized enzyme. (who.int)
Protein1
  • Intriguingly, when both bNLS and NLS (residues 320-405) were present, the fused -galactosidase protein did not accumulate in the nucleus. (who.int)
Structural1
  • Structural analysis of Saccharomyces cerevisiae alpha-galactosidase and its complexes with natural substrates reveals new insights into substrate specificity of GH27 glycosidases.Fernandez-Leiro R., Pereira-Rodriguez A., Cerdan M.E., Becerra M., Sanz-Aparicio J.J. Biol. (joplink.net)
Beta2
  • For detailed information about blood-group-substance endo-1,4-beta-galactosidase, go to the full flat file . (brenda-enzymes.org)
  • UNIQUE FORMULA - supplies the added support of alpha- galactosidase and beta-glucanase to break down vegetables, beans and grains. (haarp.net)
Diet2
  • Oral α-galactosidase taken with high G OS foods provides a clinically significant reduction in symptoms in GOS-sensitive individuals with IBS, and this strategy can be translated into practice to improve tolerance to high GOS foods as an adjunct therapy to the low FODMAP diet. (semanticscholar.org)
  • Even with the uncontrolled diet, intolerant subjects treated with DDM Galactosidase exhibited reduced gut symptoms significantly better than the control treatment as well as having a diet devoid of offending foods. (semanticscholar.org)
Levels1
  • Diagnosis was confirmed by renal biopsy, GLA mutation and low alpha-galactosidase A levels. (bmj.com)
High1
  • 3. Alpha galactosidase enzyme maintains high stability during feed storage. (bestzyme.com)
Active1
  • Alpha-galactosidase versus active charcoal for improving sonographic visualization of abdominal organs in patients with excessive intestinal gas. (semanticscholar.org)
Treatment1
  • Although larger and longer trials are needed to confirm this result, α-galactosidase seems to be a safe, well tolerated and effective treatment for gas-related symptoms in the pediatric population. (semanticscholar.org)
Amylase5
  • Bestzyme Bio-Engineering Co., Ltd. is a professional enzyme company, we provide china enzyme, Alpha amylase and etc. (kitairu.net)
  • 4.00% *alpha-Amylase (Aspergillus oryzae) (min). (lambertvetsupply.com)
  • not less than 100 Million CFU/g (Bifidobacterium bifidum) a Contains a source of alpha-amylase which can hydrolyze starch 1 USP unit of amylase activity = amount of pancreatin that dissolves starch under standard conditions. (lambertvetsupply.com)
  • Amylase 4000 DU Protease 4.5 10,000 HUT Alpha-galactosidase 50 Gal U Phytase 5 FTU Lipase 100 FIP Lysozyme (from egg white) 2000 MCG Cellulase 100 CU Lecithin Granules 450 mg. (homevet.com)
  • The pancreas and salivary gland make amylase (alpha amylase) to hydrolyse dietary starch into disaccharides and trisaccharides which are converted by other enzymes to glucose to supply the body with energy. (drbillspetnutrition.ca)
Lipase1
  • Dietary restriction has shown promise for disorders such as lysosomal acid lipase deficiency (Wolman disease), as has incorporation of lipid-lowering drugs in the regimen along with sebelipase alpha, a recombinant enzyme replacement therapy. (medscape.com)
Lactase1
  • [17] Lactose is normally cleaved in the small intestine by an enzyme called lactase (also known as β-galactosidase). (allergyresearchgroup.com)
Invertase2
  • The formula supplies the added support of alpha-galactosidase and beta-glucanase to break down vegetables, beans and grains, and enzymes such as glucoamylase and invertase to assist with the digestion of sugars. (allstarhealth.com)
  • I am now taking Muccostop with xylanase , invertase, hemicellulase, mucolase, alpha galactosidase etc between meals on an empty stomach. (curezone.com)
Beta-glucanase1
  • Dezember 2013 zur Zulassung einer Zubereitung von alpha-Galactosidase aus Saccharomyces cerevisiae (CBS 615.94) und Endo-1,4-beta-Glucanase aus Aspergillus niger (CBS 120604) als Zusatzstoff in Futtermitteln für. (efta.int)
Enzymes5
  • The enzymes responsible for hydrolyzing galactosidic linkages are termed as galactosidases. (springeropen.com)
  • Humans have two enzymes with α-galactosidase activity and an acidic pH optimum, α-N-acetylgalactosaminidase (α-NAGA) (previously called α-galactosidase B) and α-galactosidase A (α-GAL). (biomedcentral.com)
  • Digestive enzymes like those found in Digesta Alpha improve gastrointestinal (GI) functioning and better health. (digestahealth.com)
  • One of the most popular carbohydrase enzymes is alpha-galactosidase. (deerland.com)
  • PMID- 214397 TI - Influence of alpha-naphthylthiourea on blood glucose & some enzymes in albino rats. (nih.gov)
1.222
  • The α-galactosidases (α-D-galactosidegalactohydrolase, EC 3.2.1.22) are carbohydrases that catalyze the hydrolysis of α-galactosidic linkages in galacto-oligosaccharides such as raffinose, melibiose, stachyose, verbascose and ajugose, polysaccharide-like galactomannans, and glycoconjugates such as glycoproteins and glycolipids. (springeropen.com)
  • Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). (nih.gov)
Gene10
  • in the second tier, patients with the lowest alpha-galactosidase levels were further subjected to mutation analysis of the GLA gene. (medscape.com)
  • Indicated for adults in whom a diagnosis of Fabry disease has been confirmed and who possess an amenable galactosidase-α gene ( GLA ) variant (as determined through in vitro assay data). (medscape.com)
  • Multifocal white matter lesions associated with the D313Y mutation of the α-galactosidase A gene. (scielo.br)
  • The GLA gene provides instructions for making an enzyme called alpha-galactosidase A. This enzyme is active in lysosomes, which are structures that act as recycling centers within cells. (nih.gov)
  • The most common type of mutation changes a single protein building block (amino acid) in alpha-galactosidase A. Other mutations delete part of the GLA gene, insert extra genetic material into the gene, or insert a premature stop signal in the gene's instructions for making alpha-galactosidase A. Alterations in the GLA gene produce an abnormal version of the enzyme that is unable to break down globotriaosylceramide effectively. (nih.gov)
  • This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. (antikoerper-online.de)
  • The cause of FD is a variety of mutations in the GLA gene on the X chromosome (Xq22.1), resulting in a deficiency of the lysosomal enzyme alpha-galactosidase A (AGAL). (skintherapyletter.com)
  • A mutation in a gene called alpha-galactosidase A , or GLA, leads to deficient levels of the alpha-galactosidase A enzyme, which allows the fat to accumulate. (the-scientist.com)
  • Fabry disease is an X-linked storage disease due to mutations in the GLA gene encoding the lysosomal enzyme α -galactosidase A, leading to the accumulation of enzyme substrates, namely, globotriaosylceramide (Gb3), lyso-globotriaosylceramide (lyso-Gb3), and galabiosylceramide [ 1 ]. (hindawi.com)
  • Fabry disease is caused by mutations in the GLA gene, which result in absent or markedly reduced activity of the alpha-galactosidase A (Gal A) enzyme. (fabrydiseasenews.com)
Lactose3
  • 6. The medium as claimed in claim 5, wherein said substrate for the metabolic activity is specific for E. coli and is selected from a substrate for beta-glucuronidase, beta-galactosidase or alpha-galactosidase, for acidification of lactose, or for tryptophanase, beta-ribosidase, phosphatase, L-alanine aminopeptidase or L-leucine aminopeptidase. (freepatentsonline.com)
  • Beta-galactosidase -- The enzyme that splits lactose into glucose and galactose. (nih.gov)
  • The steady state values, attained upon simulation of model equations, for Allolactose (A), mRNA (M), beta-galactosidase (B), Lactose (L), and Permease (P) match with those predicted by the paper. (nih.gov)
Deficiency of alpha-galactosidase2
  • Agalsidase beta is used in the treatment of Fabry disease (a deficiency of alpha-galactosidase A enzyme). (uofmhealth.org)
  • It generates a dysfunction of glycosphingolipid metabolism due to an enzymatic deficiency of alpha-galactosidase activity, resulting in glycosphingolipid deposits in all areas of the body. (unibe.ch)
Blend1
Fabry Disease10
  • Anderson-Fabry disease (AFD) is an X-linked condition originating from a deficiency in alpha-galactosidase, a lysosomal enzyme. (medscape.com)
  • Anderson-Fabry disease (AFD) originates from a deficiency in alpha-galactosidase A (AGALA), a lysosomal enzyme. (medscape.com)
  • Mutations that eliminate the activity of the alpha-galactosidase A enzyme lead to the severe, classic form of Fabry disease, which typically begins in childhood. (nih.gov)
  • The molecular defect leading to Fabry disease: structure of human alpha-galactosidase. (nih.gov)
  • Matsuzawa F, Aikawa S, Doi H, Okumiya T, Sakuraba H. Fabry disease: correlation between structural changes in alpha-galactosidase, and clinical and biochemical phenotypes. (nih.gov)
  • Background Fabry disease (α-galactosidase deficiency) is an X-linked genetic disease caused by a variety of pathogenic GLA variants. (bmj.com)
  • In males, alpha-galactosidase assays are used to diagnose Fabry disease. (medscape.com)
  • Because levels of Gb 3 and alpha-galactosidase A may be normal in female (heterozygote) patients, genetic and/or molecular diagnosis is necessary to confirm Fabry disease in female patients with clinical features. (medscape.com)
  • Fenetically engineered, glycosylated human alpha-galactosidase isoenzyme A used in treatment of Fabry disease. (nih.gov)
  • Enzyme replacement in Fabry disease: treatment of cultured skin fibroblasts with a purified alpha-galactosidase from ficin. (utmb.edu)
Protein1
  • Among his feats, Subramaniam was able to visualize the beta-galactosidase protein with single-particle cryo-EM at an average spatial resolution of 0.22 nanometers-a level of near-atomic detail once considered the exclusive purview of X-ray crystallography. (nih.gov)
Beano1
  • Beano contains alpha galactosidase, an enzyme that is taken before meals, to break down complex carbohydrates in food to reduce gas production. (gas-x.com)
Escherichia3
  • Analysis of melibiose mutants deficient in alpha-galactosidase and thiomethylgalactoside permease II in Escherichia coli K-12. (wikidata.org)
  • Regulation of enzymatic activity in the intact cell: the beta-D-galactosidase of Escherichia coli. (wikidata.org)
  • Galactose stimulation of beta-galactosidase induction in galactokinaseless mutants of Escherichia coli. (wikidata.org)
Trichoderma2
  • Nutrizyme®AG2, alfa-galactosidase er fremstillet af Trichoderma spp. (enzymes.bio)
  • galactosidase A in the filamentous fungus Trichoderma reesei. (antikoerper-online.de)
Catalytic1
  • The monomer folds into a catalytic (alpha/beta)(8) barrel and a C-terminal beta-sandwich domain with unassigned function. (nih.gov)
Lipoic Acid2
  • Please note that 25% off applies to single bottles only and cannot be used in conjunction with multipack offers Antioxidant and Free Radical Neutralizer Alpha-Lipoic Acid™ is a 'network' antioxidant that has been studied for its potential abilities in the repair of oxidative damage, regeneration of other antioxidants, anti-inflammatory properties and. (goodhealthusa.com)
  • It also contains the protective nutrients alpha lipoic acid, NAC, inositol and choline to complement the neural, cognitive, cardiovascular, liver and skin health properties of this diverse profile. (healthywithjodi.com)
Amenable1
  • Migalastat is a pharmacological chaperone that is designed to selectively and reversibly bind with high affinity to the active sites of certain mutant forms of alpha-Gal A, the genotypes of which are referred to as amenable mutations. (nih.gov)
Starches1
Digestion2
  • An essential enzyme for healthy digestion, Alpha Galactosidase helps to breakdown carbohydrates commonly found in plants to help prevent bloating and discomfort. (spicefruit.com)
  • Alpha Galactosidase is an enzyme that increases the breakdown of galactoside in order to improve digestion and reduces bloating, discomfort, and flatulence caused by gas. (dinosaurnutritionusa.com)
Enzyme replace1
  • Neuropathic pain agents, enzyme replacement therapy (ERT), or α-galactosidase A (α-Gal A) stabilizers may be indicated. (medscape.com)
Deficient1
  • GLA -coding region mutations that cause deficient α-galactosidase A (αGLA) enzyme activity may result in this disease ( 1 1. (scielo.br)
Carbohydrates3
  • Alpha-galactosidase help digest carbohydrates. (naturalfoodseries.com)
  • This dual-action formula also includes Alpha Galactosidase to help your body break down carbohydrates to help prevent bloating and discomfort. (spicefruit.com)
  • Unlike many other gas medicines, Alpha Galactosidase can help prevent gas before it starts by breaking down the complex carbohydrates found in gassy foods into simpler, more easily digestible sugars. (drguberman.com)
Aspergillus1
Galactose3
  • Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. (uniprot.org)
  • α-galactosidases and β-galactosidases depending on the configuration of the anomeric carbon atom of galactose in the substrate molecule on which they act. (springeropen.com)
  • Alpha-Galactosidase ist eine Exoglycosidase, die α-D-Galactose spaltet und zahlreiche Anwendungen in der Medizin-, Biotechnologie-, Lebensmittel- und Pharmaindustrie hat. (eapb.org)
Globotriaosylceramide2
  • Binding to and stabilizing endogenous α-galactosidase A (α-Gal A), migalastat chaperones the enzyme from the endoplasmic reticulum to lysosomes, where α-Gal A can degrade the accumulated glycolipid (globotriaosylceramide [GL-3]) and globotriaosylsphingosine (lyso-Gb3). (medscape.com)
  • Alpha-galactosidase A breaks down a molecule called globotriaosylceramide, which consists of three sugars attached to a fatty substance. (nih.gov)
Saccharomyces1
  • The crystal structures of tetrameric Saccharomyces cerevisiae alpha-galactosidase and its complexes with the substrates melibiose and raffinose have been determined to 1.95, 2.40, and 2.70 A resolution. (nih.gov)
Characterization1
  • High-yield production and biochemical characterization of α-galactosidase produced from locally isolated Penicillium sp. (springeropen.com)
Lack the enzyme2
  • The thing about raffinose is that it passes through the small intestine undigested because we lack the enzyme alpha-galactosidase. (diabeticmediterraneandiet.com)
  • Humans lack the enzyme to break down GOS, alpha-galactosidase. (fanaticcook.com)
Substrate2
  • K-m and V-max using p-nitrophenyl-alpha-D-galactopyranoside (PNPG) as substrate were 1.07 mM and 0.01 U/mg, respectively. (ege.edu.tr)
  • Coformulation of a Novel Human α-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice. (antikoerper-online.de)
Active ingredient1
Breakdown1
  • Chapter 4, the Alpha Galactosidase breakdown data are shown at the regional level, to show the sales, revenue, and growth by regions. (pharmiweb.com)
Bacteria1
  • α-Galactosidase from bacteria, especially probiotic bacteria like bifido and lactic acid bacteria are used as "live cultures" in fermented soymilk for removal of RFOs or in the production of α-galactosidases (Farzadi et al. (springeropen.com)
Units1
  • Alpha-galactosidases catalyze the hydrolysis of terminal alpha-1,6-galactosyl units from galacto-oligosaccharides and polymeric galactomannans. (nih.gov)
Extract1
  • The highest product yield of α-galactosidase was obtained with media containing yeast extract (6.672 U/ml) as organic nitrogen and ammonium nitrate (6.325 U/ml) and as inorganic nitrogen source with media pH 5.5, and the time course of enzyme production was at the 5th day of fermentation, respectively. (springeropen.com)
Galactomannans1
  • 1997 ). α-Galactosidase is also used to improve the gelling properties of galactomannans to be used as food thickeners (Chen and Mustapha 2012 ). (springeropen.com)
A-Z Drug Index for Prescription and OTC Medications
A-Z Drug Index for Prescription and OTC Medications (medindia.net)
Dose-Ranging Study of ST-920, an AAV2/6 Human Alpha Galactosidase A Gene Therapy in Subjects With Fabry Disease - Full Text...
Dose-Ranging Study of ST-920, an AAV2/6 Human Alpha Galactosidase A Gene Therapy in Subjects With Fabry Disease - Full Text... (clinicaltrials.gov)
MedlinePlus: Genes: G
MedlinePlus: Genes: G (medlineplus.gov)
Fabry disease: MedlinePlus Genetics
Fabry disease: MedlinePlus Genetics (medlineplus.gov)
Digestive Enzymes and Weight Loss: Do They Help?
Digestive Enzymes and Weight Loss: Do They Help? (healthline.com)
Natural and Recombinant - Human 1 ELISA - TELOS The Electronic Library of Life Science
Natural and Recombinant - Human 1 ELISA - TELOS The Electronic Library of Life Science (telospub.com)
Recombinant Macaca mulatta Interleukin-1 alpha (IL1A) | CSB-YP011613MOW | Cusabio
Recombinant Macaca mulatta Interleukin-1 alpha (IL1A) | CSB-YP011613MOW | Cusabio (joplink.net)
Rainbow Light Active Adult 50+ on sale at AllStarHealth.com
Rainbow Light Active Adult 50+ on sale at AllStarHealth.com (allstarhealth.com)
Human Genome Variation Society
Human Genome Variation Society (hgvs.org)
Fabry disease: Symptoms, causes, and treatment
Fabry disease: Symptoms, causes, and treatment (medicalnewstoday.com)
Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry | Journal...
Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry | Journal... (jmg.bmj.com)
Information on EC 4.1.1.50 - adenosylmethionine decarboxylase - BRENDA Enzyme Database
Information on EC 4.1.1.50 - adenosylmethionine decarboxylase - BRENDA Enzyme Database (brenda-enzymes.org)
Giddyap Girls BioEZ - KVSupply.com
Giddyap Girls BioEZ - KVSupply.com (kvsupply.com)
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Boisbriand, Canada Clinical Research Trials | CenterWatch
Boisbriand, Canada Clinical Research Trials | CenterWatch (centerwatch.com)
Angiokeratoma Corporis Diffusum (Fabry Disease) Clinical Presentation: History, Physical Examination, Complications
Angiokeratoma Corporis Diffusum (Fabry Disease) Clinical Presentation: History, Physical Examination, Complications (emedicine.medscape.com)
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Newborn Screening Codes (lhncbc.nlm.nih.gov)
Medscape | Nephrol Dial Transplant - Content Listing
Medscape | Nephrol Dial Transplant - Content Listing (medscape.com)
Fast Five Quiz: Fabry Disease Presentation and Diagnosis
Fast Five Quiz: Fabry Disease Presentation and Diagnosis (medscape.com)
(emedicine.medscape.com)
Write a review about Full Spectrum Digest with Glutalytic 90 capsules
Write a review about Full Spectrum Digest with Glutalytic 90 capsules (acuatlanta.net)
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Gastrointestinal/Digestive (pureformulas.com)
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Fabry disease - About the Disease - Genetic and Rare Diseases Information Center (rarediseases.info.nih.gov)
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Fabry disease - Getting a Diagnosis - Genetic and Rare Diseases Information Center (rarediseases.info.nih.gov)