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.
An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids.
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.
A family of galactoside hydrolases that hydrolyze compounds with an O-galactosyl linkage. EC 3.2.1.-.
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.
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)
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.
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.
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.
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.
A polyether antibiotic which affects ion transport and ATPase activity in mitochondria. It is produced by Streptomyces hygroscopicus. (From Merck Index, 11th ed)
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)
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.
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.
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.
Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.
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.
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 is a basic helix-loop-helix transcription factor that is regulated by OXYGEN availability and is targeted for degradation by VHL TUMOR SUPPRESSOR PROTEIN.
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.
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.
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.
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.
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 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.
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.
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.
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.
A vaccine consisting of DIPHTHERIA TOXOID; TETANUS TOXOID; and whole-cell PERTUSSIS VACCINE. The vaccine protects against diphtheria, tetanus, and whooping cough.
Vaccines or candidate vaccines used to prevent infection with ROTAVIRUS.
Schedule giving optimum times usually for primary and/or secondary immunization.
Two or more vaccines in a single dosage form.
Combined vaccines consisting of DIPHTHERIA TOXOID; TETANUS TOXOID; and an acellular form of PERTUSSIS VACCINE. At least five different purified antigens of B. pertussis have been used in various combinations in these vaccines.
A species of GRAM-POSITIVE ENDOSPORE-FORMING BACTERIA in the family BACILLACEAE, found in soil, hot springs, Arctic waters, ocean sediments, and spoiled food products.
A family of bacteria which produce endospores. They are mostly saprophytes from soil, but a few are insect or animal parasites or pathogens.
A genus of gram-positive, endospore-forming, thermophilic bacteria in the family BACILLACEAE.
The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.
A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.
A bibliographic database that includes MEDLINE as its primary subset. It is produced by the National Center for Biotechnology Information (NCBI), part of the NATIONAL LIBRARY OF MEDICINE. PubMed, which is searchable through NLM's Web site, also includes access to additional citations to selected life sciences journals not in MEDLINE, and links to other resources such as the full-text of articles at participating publishers' Web sites, NCBI's molecular biology databases, and PubMed Central.
A publication issued at stated, more or less regular, intervals.
"The business or profession of the commercial production and issuance of literature" (Webster's 3d). It includes the publisher, publication processes, editing and editors. Production may be by conventional printing methods or by electronic publishing.
The premier bibliographic database of the NATIONAL LIBRARY OF MEDICINE. MEDLINE® (MEDLARS Online) is the primary subset of PUBMED and can be searched on NLM's Web site in PubMed or the NLM Gateway. MEDLINE references are indexed with MEDICAL SUBJECT HEADINGS (MeSH).
The vapor state of matter; nonelastic fluids in which the molecules are in free movement and their mean positions far apart. Gases tend to expand indefinitely, to diffuse and mix readily with other gases, to have definite relations of volume, temperature, and pressure, and to condense or liquefy at low temperatures or under sufficient pressure. (Grant & Hackh's Chemical Dictionary, 5th ed)
Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).
A vascular, horny neoplasm of the skin characterized by TELANGIECTASIS and secondary epithelial changes including acanthosis and hyperkeratosis.
A subspecialty of pathology concerned with the molecular basis (e.g., mutations) of various diseases.
A plant genus in the family PINACEAE, order Pinales, class Pinopsida, division Coniferophyta. They are coniferous evergreen trees with long, flat, spirally arranged needles that grow directly from the branch.
Glycosphingolipids which contain as their polar head group a trisaccharide (galactose-galactose-glucose) moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in ceramide trihexosidase, is the cause of angiokeratoma corporis diffusum (FABRY DISEASE).
Ocular disorders attendant upon non-ocular disease or injury.

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)

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 ...
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...
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 ...
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 ...
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 ...
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:
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] ...
Learn about Fabrazyme (Agalsidase Beta) may treat, uses, dosage, side effects, drug interactions, warnings, patient labeling, reviews, and related medications.
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.
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.
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 ...
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
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 ...
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.
Purchase high purity enzyme alpha-Galactosidase (Aspergillus niger) for use in research, biochemical enzyme assays and in vitro diagnostic analysis.
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 ...
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. ...
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...
Beano Drops information about active ingredients, pharmaceutical forms and doses by GlaxoSmithKline, Beano Drops indications, usages and related health products lists
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 ...
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.. ...
FULL SPECTRUM DIGEST 30 VEGCAPS-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. Supplemen
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[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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POSTDOCTORAL POSITIONS AVAILABLE My laboratory is investigating various aspects of how the immune system carries out surveillance to detect viral infections, cancers and cell death. Among the areas of research are: (1) The alarm signals and the receptors that alert the immune system to potential danger; (2) The mechanisms by which sentinel cells (dendritic cells) acquire and display antigens to CD8 T cells (cross presentation), a process that is essential for immune surveillance of tissues; and, (3) The antigen presentation pathway by which virally infected or cancer cells display their antigens to effector CD8 T cells (MHC class I antigen presentation), a process that is essential for the immune system to detect and eliminate these pathological cells. The laboratory is in a new state of the art research building and part of a very strong and interactive immunology community at UMass Medical School. UMass Medical School is located in Worcester Massachusetts, just outside of Boston. Interested ...
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 ...
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 ...
Journal: EJNMMI - European Journal of Nuclear Medicine and Molecular Imaging ArticleTitle: Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
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 ...
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 ...
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 ...
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, ...
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 ...
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 ...
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 ...
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 ...
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 ...
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 ...
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 ...
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:. ...
Allcosmeticsource.com alpha galactosidase 2000u/g,5kg/bag,free shipping [EP170508006]- alpha galactosidase 2000u/g,1kg/bag,free shipping What is alpha galactosidase 2000u/g alpha galactosidase is an enzyme used to hydrolyze or break α-1, 6-glycosidic bonds into galactosyl oligosaccharides (α-galactosides), liberating simpler, more usable sugars and eliminating its anti-nutrient effect. Thus, it improves the utilization of the energy and proteins in feedstuff. Function of alpha galactosidase 2000u/g Hydrolyzing
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
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 …
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 ...
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 ...
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 ...
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 ...
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. ...
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 ...
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] ...
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 ...
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 ...
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 ...
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). ...
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
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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.
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 ...
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 ...
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 ...
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:. ...
Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal, cardiac and stroke clinics, 1995-2017 ...
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. ...
6-phospho-alpha-glucosidase (EC 3.2.1.122); alpha-galactosidase (EC 3.2.1.22). 6-phospho-alpha-glucosidase requires both NAD(H ... dependent 6-phospho-alpha-glucosidase. Assignment to family 4 of the glycosylhydrolase superfamily". The Journal of Biological ...
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): ...
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- ...
"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 ...
... 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 ...
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, ...
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. ...
doi:10.1007/s12257-010-0069-0. Dey PM1, Patel S, Brownleader MD (June 1993). "Induction of alpha-galactosidase in Penicillium ...
A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". The Journal of Family ...
Alpha-galactosidase (GH27), an enzyme that breaks down certain complex sugars, is a component of Beano and other products that ... "The effect of oral alpha-galactosidase on intestinal gas production and gas-related symptoms". Digestive Diseases and Sciences ...
... pegfilgrastim sold as Neulasta alpha-galactosidase A: Fabrazyme by Genzyme alpha-L-iduronidase: (rhIDU; laronidase) Aldurazyme ...
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 ...
It contains the enzymes alpha-galactosidase (α-GAL) and invertase. It was introduced as a liquid, but that has been ... 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): ...
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 ...
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- ...
The lack of alpha-galactosidase leads to Fabry disease. A deficiency of alpha galactosidase A (a-GAL A, encoded by GLA) due to ... The first treatment for Fabry's disease to be approved by the US FDA was Fabrazyme (agalsidase beta, or alpha-galactosidase) in ... Fabry disease is an inherited lysosomal storage disorder that is caused by a deficiency of alpha-galactosidase A. This enzyme ... A person who inherits this gene does not have enough of a functioning enzyme known as alpha-galactosidase A. ...
Cloning and expression of biologically active alpha-galactosidase A as a fusion protein". "USPTO: Cloning and expression of ... biologically active alpha N-acetylgalactosaminidase". "USPTO: Cloning and expression of biologically active alpha-galactosidase ... "Cloning and expression of biologically active human alpha-galactosidase A". The Mount Sinai Hospital homepage Icahn School of ... National Institute of Diabetes and Digestive and Kidney Diseases Alpha Galactosidases A And B - Molecular and Cellular ...
... 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 deficiency. "Aspergillus ...
... ceramidetrihexoside alpha-galactosidase, trihexosylceramide alpha-galactosidase, and ceramidetrihexosidase. Brady RO, Gal AE, ... Other names in common use include trihexosyl ceramide galactosidase, ceramide trihexosidase, ...
1995). "Sixty-nine kilobases of contiguous human genomic sequence containing the alpha-galactosidase A and Bruton's tyrosine ...
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 ...
... plant cell culture expressed and a chemically modified version of the recombinant alpha-Galactosidase-A protein. Protein sub- ...
Other genetically modified pigs have had alpha galactosidase transferase knocked out and fortified with hCD46 and the hTM ... Human-alpha-1-antitrypsin,[80] which has been tested in sheep and is used in treating humans with this deficiency and ... These include alpha-amylase from bacteria, which converts starch to simple sugars, chymosin from bacteria or fungi, which clots ... "Transgenic pigs designed to express human α-galactosidase to avoid humoral xenograft rejection". J Appl Genet. 54: 293-303. ...
Supplements of the enzyme supplement alpha-galactosidase may reduce symptoms if brands containing other FODMAPs, such as polyol ...
The enzyme alpha-galactosidase now is allowed to be produced in moss bioreactors by the German Federal Institute for Drugs and ...
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 ...
In 2017 the first clinical trial phase 1 was completed testing the enzyme Alpha-galactosidase, which was produced in moss, to ... database cosmoss.org Hudson Alpha Genome Sequencing Center on flagship plant genomes Strepp, René; Scholz, Sirkka; Kruse, Sven ...
... can be broken down into its component saccharides, glucose and galactose, by the enzyme alpha-galactosidase, such as ...
... it may be helpful to take a product that contains alpha-galactosidase, such as Beano. Additionally, for individuals with ...
... may refer to: Heparanase, an enzyme Blood group B branched chain alpha-1,3-galactosidase, an enzyme This disambiguation ...
Beta-galactosidase. *Hexosaminidase. *mannosidase *alpha-Mannosidase. *beta-mannosidase. *Aspartylglucosaminidase. *Fucosidase ...
alpha-1,4-glucosidase activity. • alpha-glucosidase activity. Cellular component. • membrane. • lysosomal membrane. • lysosomal ... Acid alpha-glucosidase, also called α-1,4-glucosidase[5] and acid maltase,[6] is an enzyme (EC 3.2.1.20) that helps to break ... maltose alpha-glucosidase activity. • catalytic activity. • hydrolase activity. • carbohydrate binding. • isomaltase. • ... This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. ...
Some species of mold produce alpha-galactosidase, an anti-oligosaccharide enzyme, which humans can take to facilitate digestion ...
Galactosidases *Alpha. *Beta. *alpha-Mannosidase. *Glucuronidase *Klotho. *Hyaluronidase. *Pullulanase. *Glucosylceramidase * ...
platelet alpha granule membrane. • platelet dense granule membrane. • external side of plasma membrane. • extracellular space. ... Ligands for P-selectin on eosinophils and neutrophils are similar sialylated, protease-sensitive, endo-beta-galactosidase- ... "GMP-140, a platelet alpha-granule membrane protein, is also synthesized by vascular endothelial cells and is localized in ...
McCleary BV (November 1980). "New chromogenic substrates for the assay of alpha-amylase and (1 leads to 4)-beta-D-glucanase". ... by the species Coprinopsis Cinerea consists of seven protein strands in the shape of an enclosed tunnel called a beta/alpha ...
EC 3.2.1.48 is sucrose alpha-glucosidase. Physiology[edit]. Sucrose intolerance (also known as congenital sucrase-isomaltase ...
"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)". ...
For example, a combination of alpha-amylase and cellulase was shown to degrade polymicrobial bacterial biofilms from both in ... One of the important occurrences of glycoside hydrolases in bacteria is the enzyme beta-galactosidase (LacZ), which is involved ...
Lectins, such as concanavalin A are proteins which can bind specific alpha-D-mannose and alpha-D-glucose carbohydrate molecules ... For example, the purification of E. coli β-galactosidase is accomplished by affinity chromatography using p-aminobenyl-1-thio-β ... which serves as a good substrate analog for E.Coli-B-Galactosidase. This property allows the enzyme to bind to the stationary ...
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 ... In the cyclic form there are two anomers, named alpha and beta, since the transition from the open-chain form to the cyclic ... In the beta form, the alcohol group is in the equatorial position, whereas in the alpha form, the alcohol group is in the axial ...
The discovery that HOCl blocks induction of β-galactosidase by added lactose[44] 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 ...
... β-Galactosidase) নামৰ উসেচকৰ কোডিং অংশত সুমুৱাই দিয়া হয়। ইয়াৰ ফলত উৎসেচকটো নিষ্ক্ৰিয় হ'ব আৰু ইয়াকে কোৱা হয় অন্তৰ্ভুক্তিৰে ... INF-alpha) 9. হিৰুদিন (Hirudin) 10. অনুচক্ৰিকা বৃদ্ধি কাৰক মধুমেহ (Diabetes mellitus)। চিষ্টিক ফাইব্ৰচিচ্‌ (Cystic fibrosis)। ...
EC 6.2.1.28: 3-alpha,7-alpha-dihydroxy-5-beta-cholestanate--CoA ligase ... Beta-galactosidase (EC 3.2.1.23). *Hyaluronidase (EC 3.2.1.35). Category:EC 3.3 (act on ether bonds)Edit. *Category:EC 3.3 ...
Beta-galactosidase. *Hexosaminidase. *mannosidase *alpha-Mannosidase. *beta-mannosidase. *Aspartylglucosaminidase. *Fucosidase ...
displaystyle V={\frac {V_{max}[S]}{\alpha K_{m}+\alpha ^{\prime }[S]}}={\frac {(1/\alpha ^{\prime })V_{max}[S]}{(\alpha /\alpha ... Modeling β-galactosidase and butyrylcholinesterase". Biochimica et Biophysica Acta (BBA) - General Subjects. 1770 (5): 733-746 ... displaystyle \alpha =1+{\frac {[I]}{K_{i}}}}. α. ′. =. 1. +. [. I. ]. K. i. ′. .. {\displaystyle \alpha ^{\prime }=1+{\frac {[I ... An example of a toxic peptide is alpha-amanitin, which is found in relatives of the death cap mushroom. This is a potent enzyme ...
It is protective for β-galactosidase and neuraminidase.[6] Clinical significance[edit]. Deficiencies in this gene are linked to ... exo-alpha-sialidase activity. • serine-type carboxypeptidase activity. Cellular component. • membrane. • intracellular membrane ... 1984). "Morquio B syndrome: a primary defect in beta-galactosidase". Am. J. Med. Genet. 16 (2): 261-75. doi:10.1002/ajmg. ... 1994). "Normal serum beta-galactosidase in juvenile GM1 gangliosidosis". Pediatr. Neurol. 10 (4): 317-9. doi:10.1016/0887-8994( ...
Beta-galactosidase. *Hexosaminidase. *mannosidase *alpha-Mannosidase. *beta-mannosidase. *Aspartylglucosaminidase. *Fucosidase ...
A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". J Fam Pract 39: 441-5 ...
Beta-galactosidase. *Beta-glucosidase. *Beta-ketoacyl-ACP synthase. *Beta-secretase. *Betaine-homocysteine S-methyltransferase ... Alpha catenin. *Alpha-D-phosphohexomutase superfamily. *Alpha-Mannosidase. *Alpha-N-acetylglucosaminidase. *Alphavirus ...
alpha-galactosidase (Agalsidase alfa. *Agalsidase beta). *Glycosaminoglycan: iduronidase (Laronidase). *arylsulfatase B ( ...
Main article: Alpha-amylase. The α-amylases (EC 3.2.1.1 ) (CAS 9014-71-5) (alternative names: 1,4-α-D-glucan glucanohydrolase; ... An inhibitor of alpha-amylase, called phaseolamin, has been tested as a potential diet aid.[10] ... Park HS, Kim HY, Suh YJ, Lee SJ, Lee SK, Kim SS, Nahm DH (September 2002). "Alpha amylase is a major allergenic component in ... Like in other mammals, the pancreatic alpha-amylase AMY2 was duplicated multiple times. One event allowed it to evolve salivary ...
... (EC 3.2.1.101, exo-1,6-beta-mannanase, endo-alpha-1-,6-D-mannanase, endo-1,6-beta-mannanase, ... mannan endo-1,6-beta-mannosidase, 1,6-alpha-D-mannan mannanohydrolase) is an enzyme with systematic name 6-alpha-D-mannan ... Random hydrolysis of (1-,6)-alpha-D-mannosidic linkages in unbranched (1-,6)-mannans. References[edit]. *^ Nakajima, T.; Maitra ... Mannan endo-1,6-alpha-mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) ...
Dean KJ, Sweeley CC (October 1979). "Studies on human liver alpha-galactosidases. I. Purification of alpha-galactosidase A and ... Beta-galactosidase Migalastat, a drug targeting alpha-galactosidase Classification of α-galactosidases (according to CAZy) ... alpha-galactosidase-A deficiency): physiopathology, clinical signs, and genetic aspects]" [Fabrys disease (alpha-galactosidase ... Two recombinant forms of human alpha-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN). A mold-derived ...
Alpha-Galactosidase - Last updated on May 5, 2021. All rights owned and reserved by Memorial Sloan Kettering Cancer Center. ...
Deficiency in the lysosomal enzyme alpha-galactosidase (alpha-GAL) causes an accumulation of its substrate, which ultimately ... Deficiency in the lysosomal enzyme alpha-galactosidase (alpha-GAL) causes an accumulation of its substrate, which ultimately ... alpha-D-mannopyranose-(1-3)-[alpha-D-mannopyranose-(1-6)]alpha-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-( ... alpha-D-mannopyranose-(1-3)-[alpha-D-mannopyranose-(1-6)]alpha-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-( ...
Detailed Alpha-D-Galactosidase dosage information for adults. Includes dosages for Flatulence; plus renal, liver and dialysis ... Alpha-D-galactosidase oral drops:. Take 5 drops/problem food right before your first bite. A typical meal has 3 servings of ... Alpha-D-galactosidase oral tablet/capsule, chewable:. Chew or swallow whole 1 tablet/capsule per problem food right before your ...
... alpha-galactosidase A (alpha-Gal A; alpha-D-galactoside galactohydrolase, EC 3.2.1.22). To investigate the structure, ... Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A. D H Calhoun, D F Bishop, H S Bernstein, M Quinn ... Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A ... Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A ...
Drops; Oral; Alpha-Galactosidase 150 galu / 5 drop*Tablets; Oral; Alpha-Galactosidase 13.8 mg. ...
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 ...
Alpha-Galactosidase. Available forms, composition and doses of Beano Drops:. *Drops; Oral; Alpha-Galactosidase 150 galu / 5 ...
tr,Q02402,Q02402_9FUNG Alpha-galactosidase OS=Umbelopsis vinacea OX=44442 GN=alpha-galactosidase PE=2 SV=1 ... Name:alpha-galactosidaseImported. ,p>Information which has been imported from another database using automatic procedures.,/p ... Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, ...
An improved beta-galactosidase alpha-complementation system for molecular cloning in Bacillus subtilis.. Haima P1, van Sinderen ... The recently described beta-galactosidase alpha-complementation system for molecular cloning in Bacillus subtilis [Haima et al ... It was shown that large heterologous DNA fragments (up to at least 29 kb) could be cloned with lacZ alpha-complementing vectors ... Third, a new lacZ alpha complementing cloning vector was constructed, containing more unique target sites. ...
Alpha-galactosidase AgaB. A. 729. Geobacillus stearothermophilus. Mutation(s): 0 Gene Names: agaB. EC: 3.2.1.22. ... Crystal structure of GH36 alpha-galactosidase AgaB from Geobacillus stearothermophilus. *DOI: 10.2210/pdb4FNQ/pdb ... The molecular mechanism of the thermostable alpha-galactosidases AgaA and AgaB explained by X-ray crystallography and ... The α-galactosidase AgaA from the thermophilic microorganism Geobacillus stearothermophilus has great industrial potential ...
Wikipedia on Alpha-galactosidase deficiency (Less technical, ? quality control). UpToDate® on Alpha-galactosidase deficiency ( ... Alpha-galactosidase deficiency (Fabry disease, Fabry syndrome, Anderson-Fabry syndrome) is caused by gene variations that ... Nice Guidance on Alpha-galactosidase deficiency. Centre for Reviews and Dissemination databases -DARE & NHS EED (evaluates ... Agalsidase alpha (may not be as effective from switch studies[3]) *Pharmacological chaperones may help with specific mutations ...
Analysis of splice-site mutations of the alpha-galactosidase A gene in Fabry disease.. Lai LW1, Whitehair O, Wu MJ, OMeara M, ... Fabry disease is an X-linked disease caused by a defective lysosomal enzyme, alpha-galactosidase A, and characterized by skin ...
... alpha-Gal A), resulting in renal failure along with premature myocardial infarction and strokes. No effective treatment of this ... Fabry disease is a disorder of glycosphingolipid metabolism caused by deficiency of lysosomal alpha-galactosidase A ( ... Fabry disease is a disorder of glycosphingolipid metabolism caused by deficiency of lysosomal alpha-galactosidase A (alpha-Gal ... Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor Nat Med. ...
The enzyme alpha-galactosidase (alpha-GAL, also known as alpha-GAL A; E.C. 3.2.1.22) is responsible for the breakdown of alpha- ... a lysosomal storage disorder characterized by the buildup of alpha-galactosylated substrates in the tissues. alpha-GAL is an ... The high resolution structures of each step in the catalytic cycle will allow for improved drug design efforts on alpha-GAL and ... Defects in human alpha-GAL lead to the development of Fabry disease, ...
Unlike other gas and bloating medicines, Beano contains a natural digestive enzyme that can help prevent gas from beans before it starts.
Clinical characteristics of patients with alpha-galactosidase A gene variants in a German multicentre cohort of early ... Clinical characteristics of patients with alpha-galactosidase A gene variants in a German multicentre cohort of early ...
The addition of 50 mM glucose did not affect the induction of alpha-galactosidase formation by galactose. alpha-Galactosidase ... Conditions of formation, purification, and characterization of an alpha-galactosidase of Trichoderma reesei RUT C-30.. S ... Trichoderma reesei RUT C-30 formed an extracellular alpha-galactosidase when it was grown in a batch culture containing lactose ... Conditions of formation, purification, and characterization of an alpha-galactosidase of Trichoderma reesei RUT C-30. ...
J. Med. 276, 1163 (1967). Identification as an a-galactosidase: J. A. Kint, Science 167, 1268 (1970). Use in enzyme replacement ... Literature References: Human a-galactosidase A produced by recombinant DNA technology in Chinese hamster ovary cells. See: R. J ... Literature References: Human a-galactosidase A produced by recombinant DNA technology in cultured human cells. See: R. F. ... CAS Name: a-Galactosidase (human clone lAG18 isoenzyme A subunit protein moiety reduced) ...
Alpha galactosidase A Antibody 19877-1-AP has been identified with IF, WB, ELISA. 19877-1-AP detected 49 kDa band in HEK-293 ... Agalsidas and Alpha-galactosidase A, Belongs to the glycosyl hydrolase 27 family. It hydrolysis of terminal, non-reducing alpha ... Alpha galactosidase A Antibody 0 Publications. Rabbit Polyclonal, Catalog number: 19877-1-AP ... HEK-293 cells were subjected to SDS PAGE followed by western blot with 19877-1-AP(Alpha galactosidase A antibody) at dilution ...
Alpha galactosidase A Antibody 66121-1-Ig has been identified with IF, IHC, WB, ELISA. 66121-1-Ig detected 49 kDa band in HeLa ... Agalsidase and Alpha-galactosidase A, belongs to the glycosyl hydrolase 27 family. It hydrolyzes terminal, non-reducing alpha-D ... Alpha galactosidase A Antibody 0 Publications. Mouse Monoclonal, Catalog number: 66121-1-Ig ,CloneNo.: 2B2C5 Featured Product ... HeLa cells were subjected to SDS PAGE followed by western blot with 66121-1-Ig(Alpha galactosidase A antibody) at dilution of 1 ...
H1 2016 Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or - Market research report and ... Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) - Pipeline Review, ... Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) - Pipeline ... Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) Overview ...
Recombinant Protein and Alpha-galactosidase Antibody at MyBioSource. Custom ELISA Kit, Recombinant Protein and Antibody are ... Alpha-galactosidase A. Alpha-galactosidase A ELISA Kit. Alpha-galactosidase A Recombinant. Alpha-galactosidase A Antibody. Also ... Alpha-galactosidase C. Alpha-galactosidase C ELISA Kit. Alpha-galactosidase C Recombinant. Alpha-galactosidase C Antibody. Also ... Alpha-galactosidase D. Alpha-galactosidase D ELISA Kit. Alpha-galactosidase D Recombinant. Alpha-galactosidase D Antibody. Also ...
Purchase high purity enzyme alpha-Galactosidase (Guar) for use in research, biochemical enzyme assays and in vitro diagnostic ... E-AGLAN - α-Galactosidase (Aspergillus niger) E-AGLANP - α-Galactosidase (Aspergillus niger) Powder E-AGALPS - α-Galactosidase ... α-D-Galactosidase from lucerne and guar seed. McCleary, B. V. (1988). "Methods in Enzymology", Volume 160, (H. Gilbert, Ed.), ... α-Galactosidase has been shown to occur in a wide range of plants and animals and to be synthesized by microorganisms. This ...
Purchase high purity enzyme alpha-Galactosidase (Aspergillus niger) for use in research, biochemical enzyme assays and in vitro ... E-AGLANP - α-Galactosidase (Aspergillus niger) Powder E-AGLGU - α-Galactosidase (Guar) E-AGALPS - α-Galactosidase (Penicillium ... α-Galactosidase and exo-β-mannanase activities occur both in endosperm and cotyledon tissue but β-mannanase occurs only in ... α-Galactosidase, β-mannanase and exo-β-mannanase have been purified to homogeneity and their separate and combined action in ...
3-galactosidase B Recombinant Protein-WP_005843041.1 (MBS1078671) product datasheet at MyBioSource, Recombinant Proteins ... Alpha-galactosidase. Roves both branched alpha-1,3-linked galactose residues of blood group B antigens and linear alpha-1,3- ... Alpha-galactosidase. Removes both branched alpha-1,3-linked galactose residues of blood group B antigens and linear alpha-1,3- ... Alpha-1,3-galactosidase B (glaB1), Recombinant Protein. ★Popular Item★ Also Known As Recombinant Bacteroides vulgatus Alpha-1,3 ...
Fabry disease is caused by mutations in the gene encoding the lysosomal hydrolase, α-Gal A. The galactosidase, alpha (GLA) gene ... Eng, CM, Guffon, N, Wilcox, WR, Germain, DP, Lee, P, Waldek, S. "Safety and efficacy of recombinant human alpha-galactosidase A ... Fabry Disease (Anderson-Fabry Disease, Alpha-Galactosidase A Deficiency, Angiokeratoma Corporis Diffusum, Ceramide ... Fabry Disease (Anderson-Fabry Disease, Alpha-Galactosidase A Deficiency, Angiokeratoma Corporis Diffusum, Ceramide ...
... , digestive enzymes, enzymes, FODMAPs, fructose intolerance, glucose isomerase, lactase, xylose isomerase ...
... caused by mutations of the alpha-galactosidase A (GLA) gene. We analyzed 15 patients carrying the GLA haplotype -10C,T [ ... Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype. Access ...
Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells. by Douglas L. Johnson et al. ... Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells.. *. Douglas L. Johnson. , Robert ... Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells.}, author={Douglas L. Johnson and ... Lysosomal α-galactosidase in endothelial cell cultures established from a fabry hemizygous and normal umbilical veins. *Lis ...
  • As a point mutation in alpha-GAL can lead to Fabry disease, we have catalogued and plotted the locations of 245 missense and nonsense mutations in the three-dimensional structure. (rcsb.org)
  • The structure of human alpha-GAL brings Fabry disease into the realm of molecular diseases, where insights into the structural basis of the disease phenotypes might help guide the clinical treatment of patients. (rcsb.org)
  • To investigate the structure, organization, and expression of alpha-Gal A, as well as the nature of mutations in Fabry disease, a clone encoding human alpha-Gal A was isolated from a lambda gt11 human liver cDNA expression library. (pnas.org)
  • Alpha-galactosidase deficiency (Fabry disease, Fabry syndrome, Anderson-Fabry syndrome) is caused by gene variations that result in deficiency in lysosomal alpha-galactosidase . (ganfyd.org)
  • Analysis of splice-site mutations of the alpha-galactosidase A gene in Fabry disease. (nih.gov)
  • Fabry disease is an X-linked disease caused by a defective lysosomal enzyme, alpha-galactosidase A, and characterized by skin lesions and multiorgan involvement, including kidney, heart, and the central nervous system. (nih.gov)
  • Fabry disease is a disorder of glycosphingolipid metabolism caused by deficiency of lysosomal alpha-galactosidase A (alpha-Gal A), resulting in renal failure along with premature myocardial infarction and strokes. (nih.gov)
  • Defects in human alpha-GAL lead to the development of Fabry disease, a lysosomal storage disorder characterized by the buildup of alpha-galactosylated substrates in the tissues. (ubc.ca)
  • alpha-GAL is an active target of clinical research: there are currently two treatment options for Fabry disease, recombinant enzyme replacement therapy (approved in the United States in 2003) and pharmacological chaperone therapy (currently in clinical trials). (ubc.ca)
  • Previously, we have reported the structure of human alpha-GAL, which revealed the overall structure of the enzyme and established the locations of hundreds of mutations that lead to the development of Fabry disease. (ubc.ca)
  • Fabry disease is an X-linked lysosomal storage disorder resulting from the deficient activity of the enzyme α-galactosidase A (α-Gal A) and the progressive accumulation of its primary glycolipid substrate, globotriaosylceramide (GL-3). (endocrinologyadvisor.com)
  • Fabry disease is caused by deficient activity of the enzyme alpha-galactosidase A and results in damage to multiple organs including the kidney, heart, and brain. (mayocliniclabs.com)
  • Alpha Galactosidase is a topic of interest for clinical research for Fabry disease pharmacological chaperone therapy which is currently under clinical trials. (verifiedmarketresearch.com)
  • For more information, see Newborn Screening Act Sheet Fabry Disease: Decreased Alpha-Galactosidase A in Special Instructions. (testcatalog.org)
  • Fabry disease is an X-linked lysosomal storage disorder resulting from deficient activity of the enzyme alpha-galactosidase A (alpha-Gal A) and the subsequent deposition of glycosylsphingolipids in tissues throughout the body, in particular, the kidney, heart, and brain. (testcatalog.org)
  • The classic form of Fabry disease occurs in males who have less than 1% alpha-Gal A activity. (testcatalog.org)
  • Measurement of alpha-Gal A activity is not generally useful for identifying carriers of Fabry disease, as many of these individuals have normal levels of alpha-Gal A. Therefore, molecular genetic analysis of the GLA gene (FABRZ / Fabry Disease, Full Gene Analysis) is recommended as the most appropriate diagnostic test to detect carriers. (testcatalog.org)
  • Fabry disease is a multisystem X-linked disorder resulting from alpha-galactosidase A (alpha-GalA) gene mutations leading to the accumulation of globotriaosylceramide mainly in endothelium compromising heart, kidney, and brain. (unifesp.br)
  • We were interested in the possible interactions between ACE inhibitors therapy and the only causative therapy for Fabry disease, the enzyme replacement therapy (ERT) using recombinant human alpha-GalA (rh alpha-GalA). (unifesp.br)
  • A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. (genecards.org)
  • A deficiency of type A α- d -galactosidase is associated with Fabry disease. (thefreedictionary.com)
  • Fabry disease is an X-linked recessive inborn metabolic disorder in which a deficiency in lysosomal enzyme α-galactosidase A (Gal A) causes the systemic accumulation of globotriaosylceramide (Gb3). (springer.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. (medlineplus.gov)
  • The molecular defect leading to Fabry disease: structure of human alpha-galactosidase. (medlineplus.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. (medlineplus.gov)
  • Study Population: 120 patients aged 20-64 with Fabry disease that have GLA mutations or alpha-galactosidase A activity associated with no residual enzyme activity and non-Fabry male controls of the same age range and the same number of non-Fabry controls. (clinicaltrials.gov)
  • Lysosomal cc-galactosidase A (alpha-Gal) is the enzyme deficient in Fabry disease (FD), an X-linked glycosphingo-lipidosis caused by pathogenic mutations affecting the GLA gene. (unl.pt)
  • Males: plasma and/or leucocyte alpha galactosidase activity (by activity assay) less than lower limit of normal according to laboratory range and one or more of the characteristic features of Fabry disease i. (clinicaltrials.gov)
  • Fabry disease: identification of 50 novel alpha-galactosidase A mutations causing the classic phenotype and three-dimensional structural analysis of 29 missense mutations. (nature.com)
  • Fabry disease: twenty novel alpha-galactosidase A mutations and genotype-phenotype correlations in classical and variant phenotypes. (nature.com)
  • Fabry disease: 45 novel mutations in the alpha-galactosidase A gene causing the classical phenotype. (nature.com)
  • Fabry disease: twenty-two novel mutations in the alpha-galactosidase A gene and genotype/phenotype correlations in severely and mildly affected hemizygotes and heterozygotes. (nature.com)
  • Functional and clinical consequences of novel alpha-galactosidase A mutations in Fabry disease. (nature.com)
  • E.C. 3.2.1.22) is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. (wikipedia.org)
  • alpha-D-galactoside galactohydrolase, EC 3.2.1.22). (pnas.org)
  • E.C. 3.2.1.22) is responsible for the breakdown of alpha-galactosides in the lysosome. (ubc.ca)
  • Global Markets Direct's, 'Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) - Pipeline Review, H1 2016', provides in depth analysis on Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) targeted pipeline therapeutics. (marketresearch.com)
  • The report provides comprehensive information on the Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22), targeted therapeutics, complete with analysis by indications, stage of development, mechanism of action (MoA), route of administration (RoA) and molecule type. (marketresearch.com)
  • Additionally, the report provides an overview of key players involved in Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) targeted therapeutics development and features dormant and discontinued projects. (marketresearch.com)
  • The intracellular alpaha-galactosidase (alpha-D-galactosidase EC 3.2.1.22) producing fungi were isolated from soil and air by enrichment culture technique, using raffinose as the inducer. (who.int)
  • Alpha-galactosidase A is a lysosomal enzyme that catalyzes the conversion of ceramide trihexoside to lactosylceramide and galactose. (grantome.com)
  • For details on α-galactosidase see Garman lab: Interconversion of lysosomal enzyme specificities . (proteopedia.org)
  • The reduced activity of the lysosomal enzyme, α-galactosidase A (α-Gal A) leads to classic early manifestations and vascular disease of the heart, kidneys, and brain. (nature.com)
  • Two recombinant forms of human alpha-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN). (wikipedia.org)
  • Two recombinant enzyme replacement therapies are available to functionally compensate for alpha-galactosidase deficiency. (wikipedia.org)
  • Agalsidase alpha and beta are both recombinant forms of the human α-galactosidase A enzyme and both have the same amino acid sequence as the native enzyme. (wikipedia.org)
  • Human a -galactosidase A produced by recombinant DNA technology in cultured human cells. (drugfuture.com)
  • Two recombinant forms of alpha-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN).Alpha-galactosidase is glycoside hydrolase enzyme that hydrolyzes the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. (alpspure.co.in)
  • Use of a polypeptide comprising an amino acid sequence having at least 77 % identity to SEQ ID NO: 1 and exhibiting alpha-galactosidase activity, or a functional fragment thereof, or a recombinant host cell expressing said polypeptide, for hydrolyzing an -D-galactoside. (lens.org)
  • A recombinant nucleic acid construct, expression cassette or vector comprising a nucleic acid sequence encoding a polypeptide comprising an amino acid sequence having at least 77 % identity to SEQ ID NO: 1 and exhibiting alpha-gal actosidase activity, or a functional fragment thereof. (lens.org)
  • The objects of this invention are new Saccharomyces cerevisiae yeast strains into which α-galactosidase gene (MEL + ) has been transferred by using recombinant DNA methods. (freepatentsonline.com)
  • The new yeast strains prepared by using recombinant DNA methods produce more α-galactosidase than naturally occurring α-galactosidase producing yeast strains. (freepatentsonline.com)
  • S zym-AGS4FO is a powder-type alpha-galactosidase preparation made from Aspergillus through advanced liquid submerged fermentation and extraction technique. (spintoband.com)
  • IMSEAR at SEARO: Purification and characterization of intracellular alpha-galactosidase from Aspergillus sp. (who.int)
  • In this study, molecular cloning studies were performed on the a-galactosidase genes of Aspergillus fumigatus IMI 385708. (metu.edu.tr)
  • U. B. Söyler, "Cloning and characterization of industrially important alpha-galactosidase genes from the human pathogen aspergillus fumigatus," M.S. - Master of Science, Middle East Technical University, 2004. (metu.edu.tr)
  • In accordance with Article 7 of Regulation (EC) No 1831/2003, an application was submitted for a new use of a preparation of alpha-galactosidase produced by Saccharomyces cerevisiae (CBS 615.94) and endo-1,4-beta-glucanase produced by Aspergillus niger (CBS 120604). (europa.eu)
  • O fungo termofílico Aspergillus fumigatus secreta as enzimas invertase (b-frutofuranosidase) e a-galactosidase (a-D-galactosídeo galactohi-drolase) que estão envolvidas na hidrólise completa dos oligossacarídeos de rafinose . (bvsalud.org)
  • A enzima a-galactosidase foi produzida em meio de cultura do fungo Aspergillus fumigatus crescido por 36 h a 42 °C em meio mineral mínimo contendo os açúcares galactose , ou melibiose , ou rafinose como fontes de carbono . (bvsalud.org)
  • Short-chain alpha-galactosides (melibiose, raffinose, stachyose), as well as the monosaccharides galactose, dulcitol, arabinose, and arabitol, also induced alpha-galactosidase activity both when they were used as carbon sources (at a concentration of 1%) in batch cultures and in resting mycelia (at concentrations in the millimolar range). (asm.org)
  • To understand how the enzyme cleaves galactose from glycoproteins and glycolipids, we also determined the structure of the complex of alpha-GAL with its catalytic product. (rcsb.org)
  • This enzyme catalyses the following chemical reaction Random hydrolysis of (1->3)-alpha-D-galactosidic linkages in Aerobacter aerogenes capsular polysaccharide Hydrolyses the galactosyl-alpha-1,3-D-galactose linkages only in the complex substrate, bringing about depolymerization. (wikipedia.org)
  • Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. (uniprot.org)
  • The addition of 50 mM glucose did not affect the induction of alpha-galactosidase formation by galactose. (asm.org)
  • alpha-Galactosidase from T. reesei RUT C-30 was purified to homogeneity from culture fluids of galactose-induced mycelia. (asm.org)
  • It was active against several alpha-galactosides (p-nitrophenyl-alpha-D-galactoside, melibiose, raffinose, and stachyose) and galactomannan (locust bean gum) and was inhibited by the product galactose. (asm.org)
  • Secreted alpha-galactosidase required for catabolic conversion of melibiose to glucose and galactose. (mybiosource.com)
  • Roves both branched alpha-1,3-linked galactose residues of blood group B antigens and linear alpha-1,3-linked galactose structures. (mybiosource.com)
  • Alpha-galactosidases are the enzymes that catalyze the hydrolysis of terminal, are non-reducing α-D-galactose residues in α-D-galactosides, and many catabolic processes, including cleaving glycoproteins and glycolipids, and polysaccharides. (verifiedmarketresearch.com)
  • alpha-Galactosidases are enzymes that participate in the hydrolysis of terminal, non-reducing alpha-D-galactose. (apolloscientific.co.uk)
  • Finally, rh alpha-GalA also inhibited ACE activity and released galactose residues from purified rabbit lung ACE dose-dependently. (unifesp.br)
  • in summary, our results suggest that rh alpha-GalA interacts with ACE and inhibits its activity, possibly by removing the galactose residues from the enzyme. (unifesp.br)
  • Hidaka M, Fushinobu S, Ohtsu N, Motoshima H, Matsuzawa H, Shoun H, Wakagi T. Trimeric crystal structure of the glycoside hydrolase family 42 beta-galactosidase from Thermus thermophilus A4 and the structure of its complex with galactose. (proteopedia.org)
  • Allergy to galactose-α-1,3-galactose (alpha-gal) is a peculiar form of food allergy generally manifesting as an anaphylactic reaction hours after mammalian meat consumption, due to the presence of specific IgE against this oligosaccharide. (elsevier.es)
  • Capsular-polysaccharide endo-1,3-alpha-galactosidase (EC 3.2.1.87, polysaccharide depolymerase, capsular polysaccharide galactohydrolase) is an enzyme with systematic name Aerobacter-capsular-polysaccharide galactohydrolase. (wikipedia.org)
  • Also known as Alpha-galactosidase (Alpha-D-galactoside galactohydrolase) (Melibiase). (mybiosource.com)
  • GLA, also named as Melibiase, Agalsidas and Alpha-galactosidase A, Belongs to the glycosyl hydrolase 27 family. (ptglab.com)
  • Also known as Alpha-galactosidase C (Melibiase C). (mybiosource.com)
  • For example, the alpha-galactosidase A protein has a molecular weight range ot 49,000 - 101,000 and no accurate amino acid sequence data is available. (grantome.com)
  • Having isolated a human alpha-galactosidase A cDNA clone, we propose to perform detailed biochemical and molecular characterization of human alpha-galactosidase A. A full-length cDNA clone will be isolated and its nucleotide sequence determined, from which the amino acid sequence of the protein will be deduced. (grantome.com)
  • GLA (Galactosidase Alpha) is a Protein Coding gene. (genecards.org)
  • galactosidase enzyme obtained was 3.3x10-4 M AND 2.26 X 10-2 ?moles/ming/g(protein) respectively. (who.int)
  • 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. (medlineplus.gov)
  • Researchers have shown how pre-clinical models that lack alpha-Gal A have increased levels of alpha-synuclein, a protein associated with Parkinson's disease. (michaeljfox.org)
  • β-galactosidase is a protein encoded by the lacZ gene of the lac operon , and it exists as a homotetramer in its active state. (wikipedia.org)
  • Studies of residual activities of mutant enzymes in many Fabry patients showed that some of them had kinetic properties similar to those for normal alpha-Gal A, but were significantly less stable, especially in conditions of neutral pH (refs. (nih.gov)
  • The high resolution structures of each step in the catalytic cycle will allow for improved drug design efforts on alpha-GAL and other glycoside hydrolase family 27 enzymes by developing ligands that specifically target different states of the catalytic cycle. (ubc.ca)
  • Alpha-amylases are digestive enzymes which hydrolyze glycosidic bonds in starch to glucose, maltose, malt triose and dextrin. (alpspure.co.in)
  • 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. (istanbul.edu.tr)
  • All the immobilized alpha-galactosidases were more thermostable than the free enzyme at 50 degrees C. The stabilities of the Sepabead EC-EA and EC-HA adsorbed enzymes treated with glutaraldehyde compared to the stability of the free enzyme were a factor of 6 for Sepabead EC-EA and 5.3 for Sepabead EC-HA. (istanbul.edu.tr)
  • Cloning of a-galactosidase genes represents a first step towards the high level expression of these enzymes in a GRAS host. (metu.edu.tr)
  • 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. (iherb.com)
  • Decreased alpha-galactosidase activity in urine and plasma. (ganfyd.org)
  • We report here that 1-deoxy-galactonojirimycin (DGJ), a potent competitive inhibitor of alpha-Gal A, effectively enhanced alpha-Gal A activity in Fabry lymphoblasts, when administrated at concentrations lower than that usually required for intracellular inhibition of the enzyme. (nih.gov)
  • Oral administration of DGJ to transgenic mice overexpressing a mutant alpha-Gal A substantially elevated the enzyme activity in some organs. (nih.gov)
  • This chapter describes the large-scale purification of α-galactosidases with high activity on galactomannan from germinated seeds of lucerne and guar employing the affinity matrix. (megazyme.com)
  • α-D-galactosidase activity and galactomannan and galactosylsucrose oligosaccharide depletion in germinating legume seeds. (megazyme.com)
  • 11. A cleaning method comprising:contacting an isolated alpha-galactosidase enzyme comprising an amino acid sequence that is at least about identical to an alpha-galactosidase of Trichoderma reesei, with an object under conditions suitable for activity of said alpha-galactosidase enzyme, to clean said object. (patentsencyclopedia.com)
  • The physiological role of alpha-galactosidase A in lysosomal degradation of certain proteins is further defined by the fact that individuals with Fabry's disease lack alpha-galactosidase A activity in their visceral tissues. (grantome.com)
  • Main activity:alpha-Galactosidase. (apolloscientific.co.uk)
  • Alpha galactosidase A activity in Parkinson's disease. (cdc.gov)
  • To test whether reduction in lysosomal enzymatic activity in PD is specific to GCase, we measured GCase, acid sphingomyelinase (deficient in Niemann-Pick disease types A and B), alpha galactosidase A (deficient in Fabry), acid alpha-glucosidase (deficient in Pompe) and galactosylceramidase (deficient in Krabbe) enzymatic activities in dried blood spots of PD patients (n = 648) and controls (n = 317) recruited from Columbia University. (cdc.gov)
  • Our results suggest that ACE activity was significantly inhibited in plasma of Fabry patients and the blood pressure level decreased just after ERT (at the end of the rh alpha-GalA infusion). (unifesp.br)
  • Interestingly, 2 weeks later, ACE activity was significantly upregulated and the plasma levels of angiotensin II increased in the patients treated with rh alpha-GalA following the elevations of ACE activity. (unifesp.br)
  • the same inhibitory effect on ACE activity was also observed in rats after rh alpha-GalA infusion. (unifesp.br)
  • Furthermore, ACE activity in CHO cells transfected with the human ACE was inhibited dose and time-dependently by rh alpha-GalA. (unifesp.br)
  • in vitro, the incubation of plasma from healthy volunteers with rh alpha-GalA significantly reduced ACE activity. (unifesp.br)
  • 1 unit of α-galactosidase activity (U) is defined as the amount of enzyme which liberates 1 micromole of paranitrophenol per minute from 10 mmol/L of paranitrophenol-α-D-galactopyranose solution at pH 5.5 and 37 degrees centigrade. (spintoband.com)
  • galactosidase (pellet) activity of the isolated fungi species varied from 405 Mu/G to 2Mu/G after 120 hours of cultivation. (who.int)
  • galactosidase activity was observed at pH 5, in both supernatant and pellet. (who.int)
  • galactosidase activity in the pellet from 3645Mu/g were used for further studies. (who.int)
  • The first tier was a determination of alpha-galactosidase A activity using a dried blood spot on filter paper, in the second tier, patients with the lowest alpha-galactosidase levels were further subjected to mutation analysis of the GLA gene. (cdc.gov)
  • increase feed digestion Definition of Activity OneENOLZYMETM α-Galactosidase unit(U)is defined as the amount of enzyme whichliberates 10mmol/L P-nitrophenyl. (spintoband.com)
  • Previous screening studies have shown that a significant proportion of end-stage renal disease ( ESRD ) patients receiving hemodialysis (HD) have unsuspected FD, and the prevalence of low alpha-galactosidase A (αGLA) enzyme activity in these patients is higher than that in the normal population . (bvsalud.org)
  • The early-onset, multi-systemic FD classical phenotype is associated with absent or severe enzyme deficiency, as measured by in vitro assays, but patients with higher levels of residual alpha-Gal activity may have later-onset, more organ-restricted clinical presentations. (unl.pt)
  • Although the Cys118 allele is associated with high residual alpha-Gal activity in vitro, it has been classified as a pathogenic mutation, mainly on the basis of theoretical arguments about the chemistry of the cysteine residue. (unl.pt)
  • A composition or a kit comprising (i) a polypeptide comprising an amino acid sequence having at least 77 % identity to SEQ ID NO: 1, or a functional derivative thereof, and exhibiting alpha-galactosidase activity, and (ii) a divalent metal ion, preferably Mn2+, and/or the co-factor NAD+ or NADP+, preferably NAD+, and/or a reducing agent, preferably dithiothreitol. (lens.org)
  • A method of producing a fermentation product from an a-galactoside containing substrate comprising contacting the substrate with a polypeptide comprising an amino acid sequence having at least 77 % identity to SEQ ID NO: 1 and exhibiting alpha-galactosidase activity, or a functional fragment thereof, or a host cell expressing said polypeptide or a cell extract thereof. (lens.org)
  • The α-galactosidase AgaA from the thermophilic microorganism Geobacillus stearothermophilus has great industrial potential because it is fully active at 338 K against raffinose and can increase the yield of manufactured sucrose. (rcsb.org)
  • Alpha-galactosidase involved in the degradation of simple oligosaccharides like melibiose, raffinose and stachyose, and of polymeric galacto(gluco)mannans. (mybiosource.com)
  • Active on paranitrophenyl-alpha-galactoside, raffinose, locust bean gum and gum guar. (mybiosource.com)
  • Baker's and distiller's yeasts producing α-galactosidase, are utilizable in the corresponding industry, because they are able to utilize the raffinose present in molasses, which results in greater yield of yeast (or ethanol) and reduction or elimination of the costs associated with biological oxygen demand (B.O.D.) in the effluent from factories. (freepatentsonline.com)
  • An improved beta-galactosidase alpha-complementation system for molecular cloning in Bacillus subtilis. (nih.gov)
  • The recently described beta-galactosidase alpha-complementation system for molecular cloning in Bacillus subtilis [Haima et al. (nih.gov)
  • Due to the fact that alpha-galactosidase A is found in very low quantities in normal human tissues and it is fairly labile, its biochemical and molecular properties are not well defined. (grantome.com)
  • The chromosomal alpha-galactosidase A gene will be isolated and its molecular structure will be defined. (grantome.com)
  • For details on β-galactosidase see Molecular Playground/Beta-galactosidase . (proteopedia.org)
  • A massa molecular da a-galactosidase determinada por SDS-PAGE foi de 54,7 kDa. (bvsalud.org)
  • The crystal structure of Umbelopsis vinacea alpha-galactosidase I, which belongs to glycoside hydrolase family 27, was determined at 2.0 A resolution. (proteopedia.org)
  • The Tetramer Structure of the Glycoside Hydrolase Family 27 alpha-Galactosidase I from Umbelopsis vinacea. (proteopedia.org)
  • Fujimoto Z, Kaneko S, Kim WD, Park GG, Momma M, Kobayashi H. The Tetramer Structure of the Glycoside Hydrolase Family 27 alpha-Galactosidase I from Umbelopsis vinacea. (proteopedia.org)
  • Results from this assay do not reflect carrier status because of individual variation of alpha-galactosidase enzyme levels. (testcatalog.org)
  • Conditions of formation, purification, and characterization of an alpha-galactosidase of Trichoderma reesei RUT C-30. (asm.org)
  • MATHEW, CPDW, Isolation and characterization of alpha-galactosidase from coconut endosperm, University of Colombo UC(MED), 1985: 288p. (who.int)
  • Mutant alpha-galactosidase A genes will also be isolated from Fabry's patients and sequenced. (grantome.com)
  • After the completion of the genome project of A. fumigatus, regions with homology to a-galactosidase genes were searched on the genome of A. fumigatus. (metu.edu.tr)
  • Two a-galactosidase genes, namely aglA and aglB were amplified with these specific primers, sequenced, and ligated to vector pUC19. (metu.edu.tr)
  • High purity α-Galactosidase (Guar) for use in research, biochemical enzyme assays and in vitro diagnostic analysis. (megazyme.com)
  • α-D-Galactosidase from lucerne and guar seed. (megazyme.com)
  • Lucerne and guar contained two α-galactosidase activities, carob three and soybean four. (megazyme.com)
  • Using an affinity purified rabbit polyclonal antibody to human alpha-galactosidase A, we have demonstrated its monospecificity and screened a human liver cDNA library constructed using the expression vector gt-11. (grantome.com)
  • In addition, immediate anaphylaxis may develop after exposure to other sources of alpha-gal, such as monoclonal antibody cetuximab, vaccines, plasma expanders or anti-snake venoms. (elsevier.es)
  • Fabry's disease: alpha-galactosidase deficiency. (proteopedia.org)
  • Fabry's disease (AFD) is an X-linked lysosomal storage disease, resulting from a deficiency in alpha-galactosidase A (AGALA). (cdc.gov)
  • α-Galactosidase (Agal) hydrolyzes the terminal α-galactosyl moiety from glycoproteins and glycolipids [1] . (proteopedia.org)
  • Trichoderma reesei RUT C-30 formed an extracellular alpha-galactosidase when it was grown in a batch culture containing lactose or locust bean gum as a carbon source. (asm.org)
  • In some particularly preferred embodiments, the isolated alpha-galactosidase enzyme comprises an amino acid sequence that is related to an alpha-galactosidase from Trichoderma reesei. (patentsencyclopedia.com)
  • 1. A cleaning composition comprising an isolated alpha-galactosidase enzyme comprising an amino acid sequence that is at least about 90% identical to an alpha-galactosidase of Trichoderma reesei. (patentsencyclopedia.com)
  • 10. The cleaning composition of claim 1, wherein said isolated alpha-galactosidase enzyme is immunologically cross-reactive with an alpha-galactosidase of Trichoderma reesei. (patentsencyclopedia.com)
  • However, the DNA probes used were shown to be homologous to the a-galactosidase gene (agl1) of Trichoderma reesei. (metu.edu.tr)
  • Hydrolyzes a variety of simple alpha-D-galactoside as well as more complex molecules such as oligosaccharides and polysaccharides. (mybiosource.com)
  • β-Galactosidase (Bgal) hydrolyzes β-galactosides into monosaccharides [2] . (proteopedia.org)
  • Inhibition of alpha galactosidase was seen by N-Bromosuccinamide, oxidation of two tryptophan residues, Modification of thyrosine residue and carbodiamide. (who.int)
  • showed that the mutant non-functional β-galactosidase was lacking in part of its N-terminus with its residues 11-41 deleted, but it may be complemented by a peptide formed of residues 3-90 of β-galactosidase. (wikipedia.org)
  • However, a mutant β-galactosidase derived from the M15 strain of E. coli has its N-terminal residues 11-41 deleted and this mutant, the ω-peptide, is unable to form a tetramer and is inactive. (wikipedia.org)
  • In this method of screening, the host E. coli strain carries the lacZ deletion mutant ( lacZΔM15 ) which contains the ω-peptide, while the plasmids used carry the lacZα sequence which encodes the first 59 residues of β-galactosidase, the α-peptide. (wikipedia.org)
  • Alpha-galactosidase is a digestive enzyme that ruptures the carbohydrates in beans into simpler sugars molecules to make them easier to digest. (verifiedmarketresearch.com)
  • This was accomplished by using the putative alpha--galactosidase A cDNA clone to probe both gene dosage and somatic cell hybrid DNA panels. (grantome.com)
  • This modulation might have profound impact on the clinical outcome of Fabry patients treated with rh alpha-GalA. (unifesp.br)
  • The present study describes the clinical and sensitization characteristics of 39 patients diagnosed with alpha-gal allergy in the hospitals of our province (Lugo, Monforte de Lemos and Burela, Spain). (elsevier.es)
  • Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells. (semanticscholar.org)
  • A putative human alpha-galactosidase A clone was identified after screening 2 million recombinants in the library. (grantome.com)
  • Identification as an a -galactosidase: J. A. Kint, Science 167, 1268 (1970). (drugfuture.com)
  • A line of best fit will be used to show the relationship between the glucose concentration and the amount of alpha galactosidase. (ipl.org)
  • see table #2) The mixture with the bean water caused the solution to not be as concentrated, limiting the amount of oligosaccharides that the alpha galactosidase can break down, therefore resulting in a small amount of glucose concentration. (ipl.org)
  • Third, a new lacZ alpha complementing cloning vector was constructed, containing more unique target sites. (nih.gov)
  • It was shown that large heterologous DNA fragments (up to at least 29 kb) could be cloned with lacZ alpha-complementing vectors carrying the replication functions of the cryptic B. subtilis plasmid pTA1060, and that these inserts were structurally stably maintained for at least 100 generations of growth. (nih.gov)
  • However, when the two peptides are expressed together, as when a plasmid containing the lacZα sequence is transformed into a lacZΔM15 cells, they form a functional β-galactosidase enzyme. (wikipedia.org)
  • Here, we present the structure of the human alpha-GAL glycoprotein determined by X-ray crystallography. (rcsb.org)
  • The functional role of glutamine-280 and threonine-282 in human alpha-galactosidase. (springer.com)
  • A. fumigatus is a thermotolerant fungus and can efficiently produce thermostable a-galactosidase. (metu.edu.tr)
  • This enzyme is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. (wikipedia.org)
  • Purified alpha galactosidase was reduced alkylated, and fragment. (who.int)
  • This phenomenon of α-complementation was first demonstrated in work done by Agnes Ullmann in the laboratory of François Jacob and Jacques Monod , where the function of an inactive mutant β-galactosidase with deleted sequence was shown to be rescued by a fragment of β-galactosidase in which that same sequence, the α-donor peptide, is still intact. (wikipedia.org)