Complement C3c
alpha-Galactosidase
Fabry Disease
An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.
Galactosidases
beta-Galactosidase
Cerebrosides
Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)
Lactose
Galactose
An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.
Acridine Orange
alpha-L-Fucosidase
Nigericin
Glycosides
Any compound that contains a constituent sugar, in which the hydroxyl group attached to the first carbon is substituted by an alcoholic, phenolic, or other group. They are named specifically for the sugar contained, such as glucoside (glucose), pentoside (pentose), fructoside (fructose), etc. Upon hydrolysis, a sugar and nonsugar component (aglycone) are formed. (From Dorland, 28th ed; From Miall's Dictionary of Chemistry, 5th ed)
Cell Aging
Histocytochemistry
Escherichia coli
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
alpha 1-Antitrypsin
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Receptors, Adrenergic, alpha
One of the two major pharmacological subdivisions of adrenergic receptors that were originally defined by the relative potencies of various adrenergic compounds. The alpha receptors were initially described as excitatory receptors that post-junctionally stimulate SMOOTH MUSCLE contraction. However, further analysis has revealed a more complex picture involving several alpha receptor subtypes and their involvement in feedback regulation.
Hypoxia-Inducible Factor 1, alpha Subunit
alpha7 Nicotinic Acetylcholine Receptor
Integrin alpha3beta1
Cell surface receptor for LAMININ, epiligrin, FIBRONECTINS, entactin, and COLLAGEN. Integrin alpha3beta1 is the major integrin present in EPITHELIAL CELLS, where it plays a role in the assembly of BASEMENT MEMBRANE as well as in cell migration, and may regulate the functions of other integrins. Two alternatively spliced isoforms of the alpha subunit (INTEGRIN ALPHA3), are differentially expressed in different cell types.
Integrin alpha4
Integrin alpha6
An integrin alpha subunit that primarily associates with INTEGRIN BETA1 or INTEGRIN BETA4 to form laminin-binding heterodimers. Integrin alpha6 has two alternatively spliced isoforms: integrin alpha6A and integrin alpha6B, which differ in their cytoplasmic domains and are regulated in a tissue-specific and developmental stage-specific manner.
Integrin alpha5beta1
Integrin alpha4beta1
Integrin alpha4beta1 is a FIBRONECTIN and VCAM-1 receptor present on LYMPHOCYTES; MONOCYTES; EOSINOPHILS; NK CELLS and thymocytes. It is involved in both cell-cell and cell- EXTRACELLULAR MATRIX adhesion and plays a role in INFLAMMATION, hematopoietic cell homing and immune function, and has been implicated in skeletal MYOGENESIS; NEURAL CREST migration and proliferation, lymphocyte maturation and morphogenesis of the PLACENTA and HEART.
Interleukin-1alpha
Integrin alpha2beta1
An integrin found on fibroblasts, platelets, endothelial and epithelial cells, and lymphocytes where it functions as a receptor for COLLAGEN and LAMININ. Although originally referred to as the collagen receptor, it is one of several receptors for collagen. Ligand binding to integrin alpha2beta1 triggers a cascade of intracellular signaling, including activation of p38 MAP kinase.
Lignocellulose degradation by Phanerochaete chrysosporium: purification and characterization of the main alpha-galactosidase. (1/449)
The main alpha-galactosidase was purified to homogeneity, in 30% yield, from a solid culture of Phanerochaete chrysosporium on 1 part wheat bran/2 parts thermomechanical softwood pulp. It is a glycosylated tetramer of 50 kDa peptide chains, which gives the N-terminal sequence ADNGLAITPQMG(?W)NT(?W)NHFG(?W)DIS(?W)DTI. It is remarkably stable, with crude extracts losing no activity over 3 h at 80 degrees C, and the purified enzyme retaining its activity over several months at 4 degrees C. The kinetics of hydrolysis at 25 degrees C of various substrates by this retaining enzyme were measured, absolute parameters being obtained by active-site titration with 2',4',6'-trinitrophenyl 2-deoxy-2, 2-difluoro-alpha-D-galactopyranoside. The variation of kcat/Km for 1-naphthyl-alpha-D-galactopyranoside with pH is bell-shaped, with pK1=1.91 and pK2=5.54. The alphaD(V/K) value for p-nitrophenyl-alpha-D-glucopyranoside is 1.031+/-0.007 at the optimal pH of 3.75 and 1.114+/-0.006 at pH7.00, indicating masking of the intrinsic effect at optimal pH. There is no alpha-2H effect on binding galactose [alphaD(Ki)=0.994+/-0.013]. The enzyme hydrolyses p-nitrophenyl beta-L-arabinopyranoside approximately 510 times slower than the galactoside, but has no detectable activity on the alpha-D-glucopyranoside or alpha-D-mannopyranoside. Hydrolysis of alpha-galactosides with poor leaving groups is Michaelian, but that of substrates with good leaving groups exhibits pronounced apparent substrate inhibition, with Kis values similar to Km values. We attribute this to the binding of the second substrate molecule to a beta-galactopyranosyl-enzyme intermediate, forming an E.betaGal. alphaGalX complex which turns over slowly, if at all. 1-Fluoro-alpha-D-galactopyranosyl fluoride, unlike alpha-D-galactopyranosyl fluoride, is a Michaelian substrate, indicating that the effect of 1-fluorine substitution is greater on the first than on the second step of the enzyme reaction. (+info)Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. (2/449)
Fabry disease is an X-linked metabolic disorder caused by a deficiency of alpha-galactosidase A (alpha-Gal A). The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide (Gb3). In patients with this disorder, glycolipid deposition in endothelial cells leads to renal failure and cardiac and cerebrovascular disease. Recently, we generated alpha-Gal A gene knockout mouse lines and described the phenotype of 10-week-old mice. In the present study, we characterize the progression of the disease with aging and explore the effects of bone marrow transplantation (BMT) on the phenotype. Histopathological analysis of alpha-Gal A -/0 mice revealed subclinical lesions in the Kupffer cells in the liver and macrophages in the skin with no gross lesions in the endothelial cells. Gb3 accumulation and pathological lesions in the affected organs increased with age. Treatment with BMT from the wild-type mice resulted in the clearance of accumulated Gb3 in the liver, spleen, and heart with concomitant elevation of alpha-Gal A activity. These findings suggest that BMT may have a potential role in the management of patients with Fabry disease. (+info)Differential expression of three alpha-galactosidase genes and a single beta-galactosidase gene from Aspergillus niger. (3/449)
A gene encoding a third alpha-galactosidase (AglB) from Aspergillus niger has been cloned and sequenced. The gene consists of an open reading frame of 1,750 bp containing six introns. The gene encodes a protein of 443 amino acids which contains a eukaryotic signal sequence of 16 amino acids and seven putative N-glycosylation sites. The mature protein has a calculated molecular mass of 48,835 Da and a predicted pI of 4.6. An alignment of the AglB amino acid sequence with those of other alpha-galactosidases revealed that it belongs to a subfamily of alpha-galactosidases that also includes A. niger AglA. A. niger AglC belongs to a different subfamily that consists mainly of prokaryotic alpha-galactosidases. The expression of aglA, aglB, aglC, and lacA, the latter of which encodes an A. niger beta-galactosidase, has been studied by using a number of monomeric, oligomeric, and polymeric compounds as growth substrates. Expression of aglA is only detected on galactose and galactose-containing oligomers and polymers. The aglB gene is expressed on all of the carbon sources tested, including glucose. Elevated expression was observed on xylan, which could be assigned to regulation via XlnR, the xylanolytic transcriptional activator. Expression of aglC was only observed on glucose, fructose, and combinations of glucose with xylose and galactose. High expression of lacA was detected on arabinose, xylose, xylan, and pectin. Similar to aglB, the expression on xylose and xylan can be assigned to regulation via XlnR. All four genes have distinct expression patterns which seem to mirror the natural substrates of the encoded proteins. (+info)Molecular cloning of a human UDP-galactose:GlcNAcbeta1,3GalNAc beta1, 3 galactosyltransferase gene encoding an O-linked core3-elongation enzyme. (4/449)
Using the full-length amino-acid sequences of the human beta1,3 galactosyltransferase (beta3GalT)-I, -II and III enzymes as query, we have identified an additional member of the beta3GalT gene family within a sequenced region of the human chromosome 21 as found in GenBank. The novel human beta3GalT-V gene included an open reading frame of 933 bp encoding a protein of 310 amino acids with a short N-terminal cytoplasmic tail, a single predicted transmembrane domain and a large lumenal catalytic domain. The human beta3GalT-V protein showed 34%, 27%, 31% and 23% sequence identity with the human beta3GalT-I, -II, -III and -IV enzymes, respectively. The expression of beta3GalT-V as a recombinant protein in Sf9 insect cells confirmed the galactosyltransferase activity catalyzed by this enzyme. Similarly to beta3GalT-I, -II and -III, the beta3GalT-V enzyme used beta-linked GlcNAc as an acceptor, but unlike the former enzymes beta3GalT-V exhibited a marked preference for the O-linked core3 GlcNAcbeta1,3GalNAc substrate. The beta3GalT-V gene was mainly expressed in human small intestine and to a lesser extent in pancreas and testis. Although beta3GalT-V transcripts were not detected in normal colon tissue, based on Northern analysis, beta3GalT-V mRNA was found in the adenocarcinoma cell line Colo 205. (+info)Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome. (5/449)
A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease. (+info)Purification and characterization of recombinant Mortierella vinacea alpha-galactosidases I and II expressed in Saccharomyces cerevisiae. (6/449)
The cDNAs coding for Mortierella vinacea alpha-galactosidases I and II were expressed in Saccharomyces cerevisiae under the control of the yeast GAL10 promoter. The recombinant enzymes purified to homogeneity from the culture filtrate were glycosylated, and had properties identical to those of the native enzymes except for improving the heat stability of alpha-galactosidase II and decreasing the specific activities of both enzymes. (+info)Cloning of the gene encoding a novel thermostable alpha-galactosidase from Thermus brockianus ITI360. (7/449)
An alpha-galactosidase gene from Thermus brockianus ITI360 was cloned, sequenced, and expressed in Escherichia coli, and the recombinant protein was purified. The gene, designated agaT, codes for a 476-residue polypeptide with a calculated molecular mass of 53, 810 Da. The native structure of the recombinant enzyme (AgaT) was estimated to be a tetramer. AgaT displays amino acid sequence similarity to the alpha-galactosidases of Thermotoga neapolitana and Thermotoga maritima and a low-level sequence similarity to alpha-galactosidases of family 36 in the classification of glycosyl hydrolases. The enzyme is thermostable, with a temperature optimum of activity at 93 degrees C with para-nitrophenyl-alpha-galactopyranoside as a substrate. Half-lives of inactivation at 92 and 80 degrees C are 100 min and 17 h, respectively. The pH optimum is between 5.5 and 6.5. The enzyme displayed high affinity for oligomeric substrates. The K(m)s for melibiose and raffinose at 80 degrees C were determined as 4.1 and 11.0 mM, respectively. The alpha-galactosidase gene in T. brockianus ITI360 was inactivated by integrational mutagenesis. Consequently, no alpha-galactosidase activity was detectable in crude extracts of the mutant strain, and it was unable to use melibiose or raffinose as a single carbohydrate source. (+info)Gene assignment in the spider monkey (Ateles paniscus chamek--APC): APE-MYH7 to 2q; AR-GLA-F8C to the X chromosome. (8/449)
Comparative gene assignment between the spider monkey species Ateles paniscus chamek (APC) and man (HSA) showed conserved syntenic associations despite extensive karyotypic rearrangement between species. Two HSA 14q genes were allocated to APC 2q, being syntenic to other HSA 14q and HSA 15q markers previously assigned to APC 2q, and to HSA 12q genes previously assigned to APC 2p. These findings were consistent with A. geoffroyi chromosome painting with human whole-chromosome probes, indicating that the genus Ateles is karyotypically very rearranged. On the other hand, three human X-linked markers were assigned to the Ateles X chromosome, indicating that this chromosome is evolutionary stable. (+info)
جامعة الأندلس الخاصة للعلوم الطبية - Agalsidase Beta (Fabrazyme)
agalsidase beta - WellSpan Health Library
Immobilization and stabilization of alpha-galactosidase on Sepabeads EC-EA and EC-HA | AVESİS
Safety and Efficacy of PRX 102 in Patients With Fabry Disease Currently Treated With REPLAGAL® (Agalsidase Alfa) - Full Text...
Keywords: agalsidase alfa efficacy enzyme replacement therapy SPTAN1 - CFTR Inhibitors for Treating Diarrheal Disease
alpha-Galactosidase 4A from Bacillus halodurans | | Apollo Scientific
Globotriaosylceramide - Wikipedia
Fabrazyme (Agalsidase Beta): Side Effects, Interactions, Warning, Dosage & Uses
Evaluation of a low dose, after a standard therapeutic dose, of agalsidase beta during enzyme replacement therapy in patients...
Molecular pathology of Fabrys disease. Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial...
English (en)
AGAW - Overview: Alpha-Galactosidase, Leukocytes
Human natural anti-alpha-galactosyl IgG. II. The specific recognition of alpha (1----3)-linked galactose residues. | Journal of...
WO 2016/097324 A1 - Alpha-galactosidases And Uses Thereof -
The Lens - Free & Open Patent and Scholarly Search
Megazyme
Pfam: He PIG assoc
What is the genotype of the EZ Competent Cells? - QIAGEN
Fabrazyme: Uses, Taking, Side Effects, Warnings - Medicine.com
Beano Drops (Alpha-Galactosidase) GlaxoSmithKline
5:03 am
Discount: Full Spectrum Digest 90 Vegcaps (A70003) By Allergy Research Group - Back Pain Information
FULL SPECTRUM DIGEST 30 VEGCAPS
Zenith Whey Protein with Enzymes for Digestion | 26g protein | Natural
Ultra Veggie Enzymes - Earthbar
GLA gene homepage - Global Variome shared LOVD
Symptoms - Alpha-gal Information
FAGPL - Specimen: Alpha-Gal Panel
AGLA • Do Print | Risikorechner
POSTDOCTORAL POSITIONS AVAILABLE
Systemic disease and the heart: Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective...
Urinary podocyte loss is increased in patients with fabry disease and correlates with clinical severity of fabry nephropathy<...
Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
Fabry Disease 2011 - Bioinformatikpedia
Difference between revisions of Fabry Disease 2011 - Bioinformatikpedia
A Study of the Safety and Efficacy of Fabrazyme in Patients With Fabry Disease - Full Text View - ClinicalTrials.gov
Central nervous system involvement in anderson-fabry diease: A clinical and MRI retrospective study | IRIS Università di Pisa
Childrens Hospital of Iowa to test new treatment for Fabry disease - University News Service - The University of Iowa
Fabry Disease Market Insights, Treatment, and Market Report by DelveInsight - Meerut Reporter
The Prevalence of Fabry Disease in Patients with Chronic Kidney Disease in Turkey: The TURKFAB Study | AVESİS
Control of proteinuria with increased doses of agalsidase alfa in a patient with Fabry disease with atypical genotype-phenotype...
OPUS Würzburg | Search
Ultrastructural deposits appearing as zebra bodies in renal biopsy: Fabry disease?- comparative case reports | BMC Nephrology...
Fabrys disease and the kidney - Renal Fellow Network
Fabry Disease - Rare Disease Quick Facts
Discount China Wholesale alpha galactosidase 2000u/g,5kg/bag,free shipping [EP170508006]- US$406.00 - Allcosmeticsource.com
PRIME PubMed | Agalsidase alfa in pediatric patients with Fabry disease: a 6.5-year open-label follow-up study
Fabry disease: guidelines for the evaluation and management of multi-organ system involvement
Fabry disease
Nanobiotechnology: Human &-Galactosidase A Mutants: Priceless Tools to DevelopNovel Therapies for Fabry Disease - IBB
Childrens Hospital Of Iowa Research Benefits Fabry Patients - University News Service - The University of Iowa
Mosaicism of podocyte involvement is related to podocyte injury in females with Fabry disease
Fabry Disease - Overview
Cardiac Microvascular Pathology in Fabry Disease | Circulation
Fabry disease - SNPedia
α-Galactosidase A - Sema4
Department of Biochemistry and Molecular Biology | UMass Amherst
High Incidence of Later-Onset Fabry Disease Revealed by Newborn Screening
Third in a series on Fabry Disease: What is the importance of imaging in Fabry d
Fabry disease in unselected patients with TIA or stroke: population-based study. - Radcliffe Department of Medicine
Shire Provides Update on Biologics License Application (BLA) Filing for REPLAGAL(R) (agalsidase alfa) With the U.S. Food and...
Alpha Galactosidase - Alpha Galactosidase Manufacturer,Supplier In Gujarat,India
First oral treatment for Fabry disease recommended for approval in the EU - Pharma World
Lack of susceptibility of cells from patients with Fabry disease to productive infection with R5 human immunodeficiency virus<...
Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with (13)C...
Fabry Disease Therapeutic Pipeline Review H1 2015: RnR Market Research
Fabrys Disease - CORE Kidney - Los Angeles, CA
Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal,...
Fabry Disease: prevalence of affected males and heterozygotes with pathogenic GLA mutations identified by screening renal,...
Alpha-galactosidase
Dean KJ, Sweeley CC (October 1979). "Studies on human liver alpha-galactosidases. I. Purification of alpha-galactosidase A and ... alpha-galactosidase-A deficiency): physiopathology, clinical signs, and genetic aspects]" [Fabry's disease (α-galactosidase-A ... β-galactosidase Migalastat, a drug targeting α-galactosidase Classification of α-galactosidases (according to CAZy) GRCh38: ... alpha-Galactosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Human GLA genome location and GLA ...
Capsular-polysaccharide endo-1,3-alpha-galactosidase
Capsular-polysaccharide+endo-1,3-alpha-galactosidase at the US National Library of Medicine Medical Subject Headings (MeSH) ... Capsular-polysaccharide endo-1,3-alpha-galactosidase (EC 3.2.1.87, polysaccharide depolymerase, capsular polysaccharide ... alpha-D-galactosidic linkages in Aerobacter aerogenes capsular polysaccharide Hydrolyses the galactosyl-alpha-1,3-D-galactose ...
Blood group B branched chain alpha-1,3-galactosidase
Heparanase (EC 3.2.1.166, Hpa1 heparanase, Hpa1, heparanase 1, heparanase-1, C1A heparanase, HPSE) is an enzyme with systematic name heparan sulfate N-sulfo-D-glucosamine endoglucanase. This enzyme catalyses the following chemical reaction endohydrolysis of (1->4)-beta-D-glycosidic bonds of heparan sulfate chains in heparan sulfate proteoglycan Heparanase cleaves the linkage between a glucuronic acid unit and an N-sulfo glucosamine unit carrying either a 3-O-sulfo or a 6-O-sulfo group. Heparanase Bame KJ (June 2001). "Heparanases: endoglycosidases that degrade heparan sulfate proteoglycans". Glycobiology. 11 (6): 91R-98R. doi:10.1093/glycob/11.6.91r. PMID 11445547. Peterson SB, Liu J (May 2010). "Unraveling the specificity of heparanase utilizing synthetic substrates". The Journal of Biological Chemistry. 285 (19): 14504-13. doi:10.1074/jbc.M110.104166. PMC 2863188. PMID 20181948. Pikas DS, Li JP, Vlodavsky I, Lindahl U (July 1998). "Substrate specificity of heparanases from human hepatoma and ...
Blood group B linear chain alpha-1,3-galactosidase
Baicalin-beta-D-glucuronidase (EC 3.2.1.167, baicalinase) is an enzyme with systematic name 5,6,7-trihydroxyflavone-7-O-beta-D-glucupyranosiduronate glucuronosylhydrolase. This enzyme catalyses the following chemical reaction baicalin + H2O ⇌ {\displaystyle \rightleftharpoons } baicalein + D-glucuronate The enzyme also hydrolyses wogonin 7-O-beta-D-glucuronide and oroxylin 7-O-beta-D-glucuronide with lower efficiency. Ikegami F, Matsunae K, Hisamitsu M, Kurihara T, Yamamoto T, Murakoshi I (November 1995). "Purification and properties of a plant beta-D-glucuronidase form Scutellaria root". Biological & Pharmaceutical Bulletin. 18 (11): 1531-4. doi:10.1248/bpb.18.1531. PMID 8593473. Zhang C, Zhang Y, Chen J, Liang X (2005). "Purification and characterization of baicalin-β-D-glucuronidase hydrolyzing baicalin to baicalein from fresh roots of Scutellaria viscidula Bge". Proc. Biochem. 40: 1911-1915. doi:10.1016/j.procbio.2004.07.003. Sasaki K, Taura F, Shoyama Y, Morimoto S (September 2000). ...
Galactosidases
If the galactoside is classified as an alpha-galactoside, the enzyme is called alpha-galactosidase, and is responsible for ... producing the alpha fragment and allowing for B-galactosidase to gain its activity. To trace the activity of B-galactosidase a ... Lack of alpha-galactosidase activity in leukocytes has been linked to Fabry Disease. Galactosidases have a variety of uses, ... the alpha-fragment gene would be inactive and the alpha fragment won't be produced. In that case B-galactosidase will not be ...
Glycoside hydrolase family 4
... alpha-galactosidase (EC 3.2.1.22); alpha-D-glucuronidase (EC 3.2.1.139). 6-phospho-alpha-glucosidase requires both NAD(H) and ... dependent 6-phospho-alpha-glucosidase. Assignment to family 4 of the glycosylhydrolase superfamily". The Journal of Biological ...
Blue-white screen
"Molecular basis of beta-galactosidase alpha-complementation". Proceedings of the National Academy of Sciences of the United ... β-galactosidase is a protein encoded by the lacZ gene of the lac operon, and it exists as a homotetramer in its active state. ... The presence of an active β-galactosidase can be detected by X-gal, a colourless analog of lactose that may be cleaved by β- ... However, a mutant β-galactosidase derived from the M15 strain of E. coli has its N-terminal residues 11-41 deleted and this ...
Beano (dietary supplement)
It contains the enzyme alpha-galactosidase (α-GAL). It was introduced as a liquid, but that has been discontinued and it is now ... Di Stefano M, Miceli E, Gotti S, Missanelli A, Mazzoccahi S, Corazza GR (January 2007). "The effect of oral alpha-galactosidase ... A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". J Fam Pract. 39 (5): ...
Glycoside hydrolase family 27
Dey PM, Pridham JB (1972). "Biochemistry of α-Galactosidases". Biochemistry of alpha-galactosidases. Adv. Enzymol. Relat. Areas ... Alpha-galactosidase is present in a variety of organisms. There is a considerable degree of similarity in the sequence of alpha ... a superfamily of alpha-galactosidases, alpha-N-acetylgalactosaminidases, and isomaltodextranases which are likely to share a ... contains a region of about 50 amino acids which is similar to a domain of the eukaryotic alpha-galactosidases. Alpha-N- ...
Glycoside hydrolase family 31
... alpha-galactosidase (EC 3.2.1.22); glucoamylase (EC 3.2.1.3), sucrase-isomaltase (EC 3.2.1.48) (EC 3.2.1.10); alpha-xylosidase ... Hermans MM, Kroos MA, van Beeumen J, Oostra BA, Reuser AJ (July 1991). "Human lysosomal alpha-glucosidase. Characterization of ... Glycoside hydrolase family 31 CAZY GH_31 comprises enzymes with several known activities; alpha-glucosidase (EC 3.2.1.20), ... Homology with the rabbit intestinal sucrase-isomaltase complex and human lysosomal alpha-glucosidase". European Journal of ...
Arthrobacter alkaliphilus
It utilizes acid phosphatase, alkaline phosphatase, alpha-galactosidase, catalase, Pyrazinamidase, and urease. It is commonly ... alpha- and beta-glucuronidase, alpha-glucosidase, alpha-mannosidase, pyrazinamidase, pyrrolidonyl arylamidase, and urease. This ...
Globoside
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, ...
Globotriaosylceramide
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. ...
Penicillium ochrochloron
Dey PM1, Patel S, Brownleader MD (June 1993). "Induction of alpha-galactosidase in Penicillium ochrochloron by guar (Cyamopsis ...
Flatulence
A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". The Journal of Family ...
Robert J. Desnick
National Institute of Diabetes and Digestive and Kidney Diseases Alpha Galactosidases A And B - Molecular and Cellular ... Cloning and expression of biologically active alpha-galactosidase A as a fusion protein, (1996). Acid sphingomyelinase gene and ... Human Development Cloning and expression of biologically active human alpha-galactosidase A, (1994). Cloning and expression of ... "Cloning and expression of biologically active human alpha-galactosidase A", published 1994-10-18, assigned to Mount Sinai ...
Aspergillus niger
Alpha-galactosidase (GH27), an enzyme that breaks down certain complex sugars, is a component of Beano and several other ... Di Stefano M, Miceli E, Gotti S, Missanelli A, Mazzocchi S, Corazza GR (January 2007). "The effect of oral alpha-galactosidase ...
List of recombinant proteins
... pegfilgrastim sold as Neulasta alpha-galactosidase A: Fabrazyme by Genzyme alpha-L-iduronidase: (rhIDU; laronidase) Aldurazyme ...
Migalastat
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 ...
Galactose-alpha-1,3-galactose
A bacterial alpha-galactosidase that efficiently removes linear alpha-gal ends from molecules has been identified. It could be ... "Identification of a GH110 subfamily of alpha 1,3-galactosidases: novel enzymes for removal of the alpha 3Gal ... Galactose-alpha-1,3-galactose, commonly known as alpha gal and the Galili antigen, is a carbohydrate found in most mammalian ... Human reaction to alpha-gal has beneficial uses as a vaccine adjuvant and for enhancing wound healing. Alpha-gal allergy ...
Streptomyces griseoloalbus
Anisha, GS; Sukumaran, RK; Prema, P (March 2008). "Evaluation of alpha-galactosidase biosynthesis by Streptomyces griseoloalbus ... Anisha, G. S.; John, Rojan P.; Prema, P.; Pandey, Ashok (4 September 2008). "Investigation on α-Galactosidase Production by ... Anisha, G. S.; Prema, P. (5 December 2006). "Production of α-galactosidase by a novel actinomycete Streptomyces griseoloalbus ... "Production and characterization of partially purified thermostable α-galactosidases from Streptomyces griseoloalbus for food ...
Glycoside hydrolase family 97
The non-catalytic domains of glycosidases from the alpha-galactosidase and alpha-glucosidase superfamilies are also ... Naumoff DG (2005). "GH97 is a new family of glycoside hydrolases, which is related to the alpha-galactosidase superfamily". BMC ... In all known glycosidases with the (beta-alpha)8-barrel fold, the amino acid residues at the active site are located on the C- ... The central part of the GH97 family protein sequences represents a typical and complete (beta/alpha)8-barrel or catalytic TIM- ...
Glycoside hydrolase family 57
... α-galactosidase (EC 3.2.1.22); amylopullulanase (EC 3.2.1.41); branching enzyme (EC 2.4.1.18). It includes a thermostable alpha ... Glycoside hydrolase family 57 CAZY GH_57 comprises enzymes with several known activities; alpha-amylase (EC 3.2.1.1), 4-alpha- ... 1993). "The purification and characterization of an extremely thermostable alpha-amylase from the hyperthermophilic ...
Fabry disease
The lack of alpha-galactosidase leads to Fabry disease. A deficiency of alpha galactosidase A (a-GAL A, encoded by GLA) due to ... Fabry disease is an inherited lysosomal storage disorder that is caused by a deficiency of alpha-galactosidase A. This enzyme ... It does so by assisting correct folding of alpha-galactosidase despite the mutations that cause Fabry disease. As of March 2022 ... A person who inherits this gene does not have enough of a functioning enzyme known as alpha-galactosidase A. ...
Catarrhini
... lost the enzyme Alpha-galactosidase, present in all other mammal lineages, sometime after the split from platyrrhini ... It is hypothesized that an ancient pathogen containing Alpha-galactosidase may be responsible, as only individuals with ... the gene for Alpha-galactosidase would have produced antibodies against the pathogen and survived. The distinction between apes ...
Aspergillus sydowii
... contains an alpha-galactosidase enzyme; this enzyme, which hydrolyses the terminal alpha-galactosyl moieties from glycolipids ... Cai GL, Lu J (2012). "Isolation and identification of a novel Aspergillus sydowii F5 Producing alpha-galactosidase and ... is used in enzyme replacement therapy to functionally compensate for genetic alpha-galactosidase A deficiency. "Aspergillus ...
Galactosylgalactosylglucosylceramidase
... ceramidetrihexoside alpha-galactosidase, trihexosylceramide alpha-galactosidase, and ceramidetrihexosidase. Brady RO, Gal AE, ... Other names in common use include trihexosyl ceramide galactosidase, ceramide trihexosidase, ...
60S ribosomal protein L36a
1995). "Sixty-nine kilobases of contiguous human genomic sequence containing the alpha-galactosidase A and Bruton's tyrosine ...
Glycoside hydrolase family 36
Alpha-galactosidase is present in a variety of organisms. There is a considerable degree of similarity in the sequence of alpha ... a superfamily of alpha-galactosidases, alpha-N-acetylgalactosaminidases, and isomaltodextranases which are likely to share a ... contains a region of about 50 amino acids which is similar to a domain of the eukaryotic alpha-galactosidases. Alpha-N- ... galactosidase from various eukaryotic species. Escherichia coli alpha-galactosidase (gene melA), which requires NAD and ...
NAGA (gene)
1989). "Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B)". Biochem. ... Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene". J. Biol. Chem. 265 (35): 21859-66. ... Warner TG, Louie A, Potier M (1991). "Photolabeling of the alpha-neuraminidase/beta-galactosidase complex from human placenta ... 1996). "Human alpha-N-acetylgalactosaminidase (alpha-NAGA) deficiency: new mutations and the paradox between genotype and ...
Enzyme inhibitor
... alpha K_{m}+\alpha ^{\prime }[S]}}={\frac {(1/\alpha ^{\prime })V_{max}[S]}{(\alpha /\alpha ^{\prime })K_{m}+[S]}}} where the ... modeling beta-galactosidase and butyrylcholinesterase". Biochimica et Biophysica Acta (BBA) - General Subjects. 1770 (5): 733- ... alpha =1+{\frac {[I]}{K_{i}}}} α ′ = 1 + [ I ] K i ′ . {\displaystyle \alpha ^{\prime }=1+{\frac {[I]}{K_{i}^{\prime }}}.} Thus ... An example of a toxic peptide is alpha-amanitin, which is found in relatives of the death cap mushroom. This is a potent enzyme ...
Hypochlorous acid
The discovery that HClO blocks induction of β-galactosidase by added lactose led to a possible answer to this question. The ... The reaction of aqueous hypochlorous acid with alpha-amino acids and dipeptides". Biochimica et Biophysica Acta. 313 (1): 170- ... "Human neutrophils employ the myeloperoxidase-hydrogen peroxide-chloride system to oxidize alpha-amino acids to a family of ...
P-selectin
McEver RP, Beckstead JH, Moore KL, Marshall-Carlson L, Bainton DF (July 1989). "GMP-140, a platelet alpha-granule membrane ... Ligands for P-selectin on eosinophils and neutrophils are similar sialylated, protease-sensitive, endo-beta-galactosidase- ...
GcMAF
List of ineffective cancer treatments Galactosidases - Advances in Research and Application. Scholarly Editions. 21 June 2013. ... misleadingly based on reduced levels of the alpha-N-acetylgalactosaminidase enzyme (also known as nagalase), whose production ...
Moss bioreactor
The enzyme alpha-galactosidase now is allowed to be produced in moss bioreactors by the German Federal Institute for Drugs and ...
Pseudogymnoascus destructans
... alpha/beta-galactosidase, β-glucuronidase, α-fucosidase, α-mannosidase, and trypsin. Important dual virulence factors found in ...
Protalix BioTherapeutics
... plant cell culture expressed and a chemically modified version of the recombinant alpha-Galactosidase-A protein. Protein sub- ...
Stem cell marker
August 2004). "Beta-galactosidase of ROSA26 mice is a useful marker for detecting the definitive erythropoiesis after stem cell ... Czerwinski M, Kiem HP, Slattery JT (March 1997). "Human CD34+ cells do not express glutathione S-transferases alpha". Gene ... October 2000). "The interleukin-3 receptor alpha chain is a unique marker for human acute myelogenous leukemia stem cells". ... asialo GM1 Bcl-2 Beta-galactosidase (β-gal) of ROSA26 mice beta1-integrin bromodeoxyuridine c-kit (CD117) c-Met C1qR(p) END ( ...
Beta-glucuronidase
Human β-glucuronidase is homologous to the Escherichia coli enzyme β-galactosidase. This homologous relationship, along with ... Alpha-glucuronidase Glucuronosyl-disulfoglucosamine glucuronidase Glycyrrhizinate beta-glucuronidase PDB: 1BHG; Jain S, ...
Galactose
A disaccharide composed of two units of galactose, galactose-alpha-1,3-galactose (alpha-gal), has been recognized as a ... The hydrolysis of lactose to glucose and galactose is catalyzed by the enzymes lactase and β-galactosidase. The latter is ... Alpha-gal allergy may be triggered by lone star tick bites. Galactose in sodium saccharin solution has also been found to cause ... In the cyclic form there are two anomers, named alpha and beta, since the transition from the open-chain form to the cyclic ...
Inulinase
... which has one alpha helix. Being made of beta sheets allows the structures to be more stable during chemical reactions. Both of ... galactosidases, glucosidases and mannosidases". J. Am. Chem. Soc. 65 (7): 1369-1380. doi:10.1021/ja01247a029. Liu Y, Zhou SH, ...
Affinity chromatography
Lectins, such as concanavalin A are proteins which can bind specific alpha-D-mannose and alpha-D-glucose carbohydrate molecules ... This property allows the enzyme to bind to the stationary phase of the affinity matrix and β-Galactosidase is eluted by adding ... For example, the purification of E. coli β-galactosidase is accomplished by affinity chromatography using p-aminobenyl-1-thio-β ...
SNCAIP
... stands for "synuclein, alpha interacting protein" and can be signified by SNCAP_HUMAN, synphilin 1, synuclein, alpha ... "Analysis of synphilin-1 and synuclein interactions by yeast two-hybrid beta-galactosidase liquid assay". Neurosci. Lett. 325 (2 ... Chung KK, Zhang Y, Lim KL, Tanaka Y, Huang H, Gao J, Ross CA, Dawson VL, Dawson TM (2001). "Parkin ubiquitinates the alpha- ... Tanaka M, Kim YM, Lee G, Junn E, Iwatsubo T, Mouradian MM (2004). "Aggresomes formed by alpha-synuclein and synphilin-1 are ...
List of diseases (N)
... folate-sensitive Neural tube defects X linked Neuraminidase beta-galactosidase deficiency Neuraminidase deficiency Neurasthenia ... also Health Exercise Nutrition N acetyltransferase deficiency N syndrome N-Acetylglutamate synthase deficiency N-acetyl-alpha-D ...
List of enzymes
3-alpha,7-alpha-dihydroxy-5-beta-cholestanate-CoA ligase EC 6.2.1.29: Transferred entry: 6.2.1.7 EC 6.2.1.30: Phenylacetate-CoA ... Beta-galactosidase (EC 3.2.1.23) Hyaluronidase (EC 3.2.1.35) Function: Amylase is an enzyme that is responsible for the ...
Snodgrassella alvi
Tests of the strains for β-glucosidase, β-galactosidase, indole production, proteolysis of gelatin, and glucose fermentation ... phylum Actinobacteria), Gilliamella apicola, Frischella perrara, Bartonella apis, and Alpha 2.1 (phylum Proteobacteria). ...
Staphylococcus nepalensis
... beta-galactosidase, and beta-glucuronidase, and hydrolyses aesculin and Tween 80. It reduces nitrate to nitrite. It is negative ... alpha- and beta-haemolysins, heat-stable and heat-labile nucleases, indole, hydrogen sulphide, and lecithinase. It produces ...
Transferase
"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)". ...
Staphylococcus muscae
... alpha- or beta-haemeolysins, ornithine decarboxylase, acetylmethylcarbinol, or beta-galactosidase. It does not hydrolyze starch ...
Beta-D-fucosidase
Chinchetru MA, Cabezas JA, Calvo P (1983). "Characterization and kinetics of beta-D-gluco/fuco/galactosidase from sheep liver ... beta-D-glucosides and alpha-L-arabinosides. ... "Beta-D-galactosidase and beta-D-fucosidase of pig kidney". ... Rodriguez JA, Cabezas JA, Calvo P (1982). "beta-Fucosidase, beta-glucosidase and beta-galactosidase activities associated in ...
Antiflatulent
... it may be helpful to take a product that contains alpha-galactosidase, such as Beano[citation needed] or kombu. Additionally, ...
GLA
... a fictional comic hero group Alpha-galactosidase, a glycoside hydrolase enzyme GLA (gene), an encoding of the enzyme alpha- ... galactosidase A Gla domain, a protein domain Gamma-Linolenic acid, a fatty acid Linde-Buzo-Gray algorithm (also called ...
Enzyme kinetics
December 1966). "The determination of the concentration of hydrolytic enzyme solutions: alpha-chymotrypsin, trypsin, papain, ... modeling beta-galactosidase and butyrylcholinesterase". Biochimica et Biophysica Acta (BBA) - General Subjects. 1770 (5): 733- ...
Dose-Ranging Study of ST-920, an AAV2/6 Human Alpha Galactosidase A Gene Therapy in Subjects With Fabry Disease - Full Text...
Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) | CSB-YP356371SAC | Cusabio
Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) from Cusabio. Cat Number: CSB-YP356371SAC. USA, UK & Europe ... Recombinant Human Alpha-galactosidase A (GLA) , CSB-EP009474HU , CusabioAlternative Name(s): Alpha-D-galactosidase AAlpha-D- ... Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) , CSB-YP356371SAC. (No reviews yet) Write a Review Write a ... Recombinant Saccharomyces cerevisiae Alpha-galactosidase 1 (MEL1) , CSB-YP356371SAC , Cusabio. Alternative Name(s): Alpha-D- ...
Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype |...
... caused by mutations of the alpha-galactosidase A (GLA) gene. We analyzed 15 patients carrying the GLA haplotype -10C>T [ ... A deficiency of alpha-galactosidase A (GLA, E.C.3.2.1.22) leads to Fabry disease (FD), an X-linked lysosomal storage disorder. ... Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype. *Michael ... Cryptogenic stroke and small fiber neuropathy of unknown etiology in patients with alpha-galactosidase A-10T genotype ...
PDF] The Effect of Oral α-Galactosidase on Intestinal Gas Production and Gas-Related Symptoms | Semantic Scholar
α-Galactosidase reduced gas production following a meal rich in fermentable carbohydrates and may be helpful in patients with ... Eight healthy volunteers ingested 300 or 1200 GalU of α-galactosidase or placebo during a test meal containing 420 g of cooked ... The aim was to evaluate the effect of α-galactosidase administration, in a randomized double-blind placebo-controlled protocol ... The administration of 1200 GalU of α-galactosidase induced a significant reduction of both breath hydrogen excretion and ...
Alpha-galactosidase - Intentional Wellness
IMSEAR at SEARO: Immobilization of alpha-galactosidase from coconut endosperm
Alpha-galactosidase was covalently bound to nylon pellets with a percentage binding of 14 percent and a retained activity of 49 ... When Alpha-galactosidase was covalently bound to nylon powder there was a percentage binding of 19 percent and a retained ... Alpha-galactosidase was also immobilized on Sepharose-4B-caproic acid with a percentage binding of 48 percent and a retained ... Purified Alpha-galactosidase (60 units) was immobilized on Sepharose-4B-capranilide gel (72 ml) in the presence of ammonium ...
A-Z Drug Index for Prescription and OTC Medications
Alpha galactosidase Alpha-d-galactosidase is an enzyme used to digest complex carbohydrates in the food and helps in digestion. ... Alpha One-proteinase inhibitor Alpha One-proteinase inhibitor is prescribed to treat alpha -1 antitrypsin deficiency, a genetic ... Alpha-linolenic acid Alpha-linolenic acid is used to prevent or treat the disease of heart and blood vessels by reducing the ... a rare genetic disorder occurs due to a deficiency of alpha-galactosidase-A (an enzyme responsible for breaking down fat). ...
MedlinePlus: Genes: G
Effects of Alpha Galactosidase Enzyme on Feed Products - BESTZYME BIO-ENGINEERING CO., LTD.
Alpha-galactosides are composed of a sucrose unit and one (or more) galactose molecules connected by α1,6 glycosidic bonds. ... Products Phytase Lipase Glucose Oxidase Beta Mannanase Xylanase Beta Glucanase Cellulase Alpha Galactosidase Pectinase Alpha ... Alpha galactosidase enzyme is the enzyme containing alpha galactosides bonds after the catalytic hydrolysis process. It can ... Alpha galactosidase enzyme is an endonuclease derived from fungal liquid submerged fermentation. The average particle size is ...
NAGA alpha-N-acetylgalactosaminidase [Homo sapiens (human)] - Gene - NCBI
Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B). N-acetylgalactosaminidase, alpha-. alpha-galactosidase B. NP_ ... the active sites of human lysosomal enzymes alpha-galactosidase and alpha-N-acetylgalactosaminidase have interconvertible ... Three dimensional structural studies of alpha-N-acetylgalactosaminidase (alpha-NAGA) in alpha-NAGA deficiency (Kanzaki disease ... enables alpha-galactosidase activity IBA Inferred from Biological aspect of Ancestor. more info ...
Fabry Disease: Symptoms, Treatment and Life Expectancy
Fabry disease is a rare genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a ... Fabry disease is a rare genetic disease with a deficiency of an enzyme called alpha-galactosidase A. The disease affects many ... Fabry disease is caused by a genetic mutation resulting in a deficiency of an enzyme called alpha-galactosidase A (a-Gal A). ... Fabry disease is the result of a mutation of the gene responsible for making alpha-galactosidase enzyme, which is located on ...
Digestive Enzymes and Weight Loss: Do They Help?
Rainbow Light Active Adult 50+ on sale at AllStarHealth.com
Human Genome Variation Society
galactosidase, alpha 300644 GLA database at LOVD Johan T. den Dunnen Leiden Univ. Med Centre (acting), Curator vacancy ... galactosidase, alpha 300644 CCHMC - Human Genetics Mutation Database Ammar Husami and Theru A. Sivakumaran ... glycine receptor, alpha 4 \N GLRA4 database at LOVD Johan T. den Dunnen Leiden Univ. Med Centre (acting), Curator vacancy ... glycine receptor, alpha 2 305990 GLRA2 database at LOVD Johan T. den Dunnen Leiden Univ. Med Centre (acting), Curator vacancy ...
Publications | Nichols Lab
Lysosomal Storage Disease: Overview, Classification of Lysosomal Storage Diseases, Glycogen Storage Disease Type II
Schindler disease results from the deficient activity of the enzyme alpha-N -acetylgalactosaminidase (alpha-galactosidase B), ... Novel sequence variants of the alpha-galactosidase A gene in patients with Fabry disease. Mol Genet Metab. 2008 Dec. 4:224-8. [ ... Evaluation of recombinant alpha-galactosidase A therapy for amelioration of the cardiovascularmanifestations of Fabry disease: ... Fabry disease (alpha-galactosidase A) (Also see the Medscape Reference article Fabry Disease.) ...
Yanhong Liu : USDA ARS
Osteocalcin Antibody (190125) (MAB1419): Novus Biologicals
Omega-Zyme Omega Zyme 180 Caplets Garden of Life - Compare Price and Reviews at VitaDigest.com
Fabry disease: Symptoms, causes, and treatment
Fabry disease results when there is a shortage of an enzyme called alpha-galactosidase A (a-GAL A). This enzyme is is encoded ... In Fabry disease patients cases, the missing enzyme is alpha-galactosidase A (a-GAL A). ... Other names include Anderson-Fabry disease, alpha-galactosidase A deficiency, or angiokeratoma corporis diffusum. ... What is 5 alpha reductase deficiency? Learn about this genetic condition, including treatment options and how it differs from ...
Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry | Journal...
Alpha-galactosidase A deficiency: Fabry disease. In: Scriver CR, Beaudet AL, Valle D, Sly WS, Childs B, Kinzler KW, Vogelstein ... Detection of 8 new mutations in the alpha-galactosidase A gene in Fabry disease. Hum Mol Genet 1994;3:667-9. doi:10.1093/hmg/ ... The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and ... Safety and efficacy of recombinant human alpha-galactosidase A--replacement therapy in Fabrys disease. N Engl J Med 2001;345:9 ...
Giddyap Girls BioEZ - KVSupply.com
Journal of Zhejiang University SCIENCE B
alpha-galactosidase induced by the constitutive PGK1 promoter was used as a food-grade selection marker. The feasibility of the ... and tumor necrosis factor-alpha (TNF-alpha). Based on these results, the anti-inflammatory effects and low cell toxicity of ... alpha-galactosidase marker was confirmed by the growth of transformants harboring the constructed vector on a medium containing ... pH had little effect on beta-glucanase or proteases partition but affected alpha-amylase partition when pH was over 7.0. The ...
Solaray Once Daily Adult 50+ on sale at AllStarHealth.com
UVigoTV - 3rd Annual Meeting Cinbio
Master Supplements Enzalase - Nutrition Depot Online
BeanAssist 30 capsules
- Bayho
Garden of Life OmegaZyme Ultra Digestive Enzymes 180 caps
KEGG REACTION: R12962
alpha-galactosidase [EC:3.2.1.22]. K07407 alpha-galactosidase [EC:3.2.1.22]. K07553 beta-1,4-galactosyltransferase 6 [EC:2.4. ... aklavinone 7-alpha-L-rhodosaminyltransferase [EC:2.4.1.326]. K15947 aklavinone 7-alpha-L-rhodosaminyltransferase auxiliary ... 1,2-diacylglycerol-3-alpha-glucose alpha-1,2-glucosyltransferase [EC:2.4.1.208]. ... phosphatidyl-myo-inositol alpha-mannosyltransferase [EC:2.4.1.345]. K09654 beta-1,3-N-acetylgalactosaminyltransferase 2 [EC:2.4 ...
DeficiencyAccumulation of glycosphingolipidsFabryRaffinoseEnzymaticHydrolaseSoybeanLysosomalHydrolysisEnzyme activityActivityPelletsProteinStructuralBetaDietLevelsHighActiveTreatmentAmylaseLipaseLactaseInvertaseBeta-glucanaseEnzymes1.22GeneLactoseDeficiency of alpha-galactosidaseBlendFabry DiseaseProteinBeanoEscherichiaTrichodermaCatalyticLipoic AcidAmenableStarchesDigestionEnzyme replaceDeficientCarbohydratesAspergillusGalactoseGlobotriaosylceramideSaccharomycesCharacterizationLack the enzymeSubstrateActive ingredientBreakdownBacteriaUnitsExtractGalactomannans
Deficiency10
- A deficiency of alpha-galactosidase A (GLA, E.C.3.2.1.22) leads to Fabry disease (FD), an X-linked lysosomal storage disorder. (biomedcentral.com)
- A new infantile case of alpha-N-acetylgalactosaminidase deficiency. (nih.gov)
- Three dimensional structural studies of alpha-N-acetylgalactosaminidase (alpha-NAGA) in alpha-NAGA deficiency (Kanzaki disease): different gene mutations cause peculiar structural changes in alpha-NAGAs resulting in different substrate specificities and clinical phenotypes. (nih.gov)
- Fabry disease is a rare genetic disease with a deficiency of an enzyme called alpha-galactosidase A. The disease affects many parts of the body including the skin, eyes, gastrointestinal system, kidney, heart , brain, and nervous system. (medicinenet.com)
- Fabry disease is a rare genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a type of fat called globotriaosylceramide (Gb3, or GL-3) in the body. (medicinenet.com)
- Fabry disease is caused by a genetic mutation resulting in a deficiency of an enzyme called alpha-galactosidase A (a-Gal A). The genetic defect causes the body to not make enough of this enzyme leading to a buildup of a type of fat called globotriaosylceramide (Gb3, or GL-3) in the body cause the symptoms of Fabry disease. (medicinenet.com)
- Other names include Anderson-Fabry disease, alpha-galactosidase A deficiency, or angiokeratoma corporis diffusum. (medicalnewstoday.com)
- Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (⍺-GalA). (biomedcentral.com)
- Dietary restriction has shown promise for disorders such as lysosomal acid lipase deficiency (Wolman disease), as has incorporation of lipid-lowering drugs in the regimen along with sebelipase alpha, a recombinant enzyme replacement therapy. (medscape.com)
- BACKGROUND AND OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal storage disease with various clinical symptoms due to a deficiency of an enzyme called alpha-galactosidase A. The likelihood of nephropathy increases with age and the severity of the mutation in Fabry patients. (bvsalud.org)
Accumulation of glycosphingolipids2
- Background Agalsidase β is a form of enzyme replacement therapy for Fabry disease, a genetic disorder characterised by low α-galactosidase A activity, accumulation of glycosphingolipids and life-threatening cardiovascular, renal and cerebrovascular events. (bmj.com)
- Fabry disease is an X-linked lysosomal storage disorder caused by reduced activity or absence of the alpha-galactosidase A enzyme resulting in systemic accumulation of glycosphingolipids. (bmj.com)
Fabry3
- Fabry disease (FD) is a multisystemic disorder with typical neurological manifestations such as stroke and small fiber neuropathy (SFN), caused by mutations of the alpha-galactosidase A ( GLA ) gene. (biomedcentral.com)
- Fabry disease is the result of a mutation of the gene responsible for making alpha-galactosidase enzyme, which is located on the X chromosome. (medicinenet.com)
- Fabry disease results when there is a shortage of an enzyme called alpha-galactosidase A (a-GAL A). This enzyme is is encoded by the GLA gene. (medicalnewstoday.com)
Raffinose2
- The purified enzyme was shown to be homogeneous by polyacrylamide gel electrophoresis and isolectric focusing.Studies on inhibition of Alpha-galactosidase by carbohydrates showed that the enzyme is no inhibited by sucrose and glucose and only 29 percent inhibited by a 1 percent raffinose solution. (who.int)
- We know of one enzyme, alpha-galactosidase, that can break down raffinose, but it isn't present in our stomachs. (zmescience.com)
Enzymatic1
- Three additional (3.4%) patients had the enzymatic activity of alpha-galactosidase slightly decreased. (biomedcentral.com)
Hydrolase1
- Alpha/beta hydrolase family [Interproscan]. (ntu.edu.sg)
Soybean1
- 6. Application scope of alpha galactosidase enzyme: soybean meal or miscellaneous meal-type pellets, concentrates and premixes of pigs, poultry and aquatic products. (bestzyme.com)
Lysosomal1
- NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. (nih.gov)
Hydrolysis3
- Alpha galactosidase enzyme is the enzyme containing alpha galactosides bonds after the catalytic hydrolysis process. (bestzyme.com)
- An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids. (bvsalud.org)
- Both saposin B and C have in vitro effects on hydrolysis of LacCer by activation of galactosylceramide-β-galactosidase, GM1-β-galactosidase and other β-galactosidases ( 10 ). (cdc.gov)
Enzyme activity1
- The disease is diagnosed with a test that measures leukocyte alpha-Gal A enzyme activity. (medicinenet.com)
Activity9
- Purified Alpha-galactosidase (60 units) was immobilized on Sepharose-4B-capranilide gel (72 ml) in the presence of ammonium sulphate with 100 percent binding and 59 percent retained activity. (who.int)
- When 7.75 units of Alpha-galactosidase was immobilized by entrapment in the K-carrageenan gel (20 ml) there was a percentage binding of 57 percent and a retained activity of 54 percent. (who.int)
- Alpha-galactosidase was covalently bound to nylon pellets with a percentage binding of 14 percent and a retained activity of 49 percent. (who.int)
- When Alpha-galactosidase was covalently bound to nylon powder there was a percentage binding of 19 percent and a retained activity of 64 percent. (who.int)
- Alpha-galactosidasewas covalently bound to Sepharose-4B-lysine with a percentage binding of 36 percent and a retained activity of 2.25 percent. (who.int)
- Alpha-galactosidase was also immobilized on Sepharose-4B-caproic acid with a percentage binding of 48 percent and a retained activity of 32 percent. (who.int)
- At 37°C and pH 5.5, the amount of enzyme required to degrade and release 1 μmol of p-nitrophenol from a 10mM concentration of p-nitrophenol αD galactopyranoside solution per minute is an α-galactosidase activity Unit (IU). (bestzyme.com)
- The five longest treated patients continued to exhibit elevated alpha-galactosidase A (α-Gal A) activity, sustained up to 15 months as of the last date of measurement. (businesswire.com)
- Alpha galactosidase A activity in Parkinson's disease. (cdc.gov)
Pellets1
- Alpha-galactosidase immobilized in the 15 percent polyacrylamide gel, K-carrageenan gel, nylon pellets and nylon power were used to study the properties of the immobilized enzyme. (who.int)
Protein1
- Intriguingly, when both bNLS and NLS (residues 320-405) were present, the fused -galactosidase protein did not accumulate in the nucleus. (who.int)
Structural1
- Structural analysis of Saccharomyces cerevisiae alpha-galactosidase and its complexes with natural substrates reveals new insights into substrate specificity of GH27 glycosidases.Fernandez-Leiro R., Pereira-Rodriguez A., Cerdan M.E., Becerra M., Sanz-Aparicio J.J. Biol. (joplink.net)
Beta2
- For detailed information about blood-group-substance endo-1,4-beta-galactosidase, go to the full flat file . (brenda-enzymes.org)
- UNIQUE FORMULA - supplies the added support of alpha- galactosidase and beta-glucanase to break down vegetables, beans and grains. (haarp.net)
Diet2
- Oral α-galactosidase taken with high G OS foods provides a clinically significant reduction in symptoms in GOS-sensitive individuals with IBS, and this strategy can be translated into practice to improve tolerance to high GOS foods as an adjunct therapy to the low FODMAP diet. (semanticscholar.org)
- Even with the uncontrolled diet, intolerant subjects treated with DDM Galactosidase exhibited reduced gut symptoms significantly better than the control treatment as well as having a diet devoid of offending foods. (semanticscholar.org)
Levels1
- Diagnosis was confirmed by renal biopsy, GLA mutation and low alpha-galactosidase A levels. (bmj.com)
High1
- 3. Alpha galactosidase enzyme maintains high stability during feed storage. (bestzyme.com)
Active1
- Alpha-galactosidase versus active charcoal for improving sonographic visualization of abdominal organs in patients with excessive intestinal gas. (semanticscholar.org)
Treatment1
- Although larger and longer trials are needed to confirm this result, α-galactosidase seems to be a safe, well tolerated and effective treatment for gas-related symptoms in the pediatric population. (semanticscholar.org)
Amylase5
- Bestzyme Bio-Engineering Co., Ltd. is a professional enzyme company, we provide china enzyme, Alpha amylase and etc. (kitairu.net)
- 4.00% *alpha-Amylase (Aspergillus oryzae) (min). (lambertvetsupply.com)
- not less than 100 Million CFU/g (Bifidobacterium bifidum) a Contains a source of alpha-amylase which can hydrolyze starch 1 USP unit of amylase activity = amount of pancreatin that dissolves starch under standard conditions. (lambertvetsupply.com)
- Amylase 4000 DU Protease 4.5 10,000 HUT Alpha-galactosidase 50 Gal U Phytase 5 FTU Lipase 100 FIP Lysozyme (from egg white) 2000 MCG Cellulase 100 CU Lecithin Granules 450 mg. (homevet.com)
- The pancreas and salivary gland make amylase (alpha amylase) to hydrolyse dietary starch into disaccharides and trisaccharides which are converted by other enzymes to glucose to supply the body with energy. (drbillspetnutrition.ca)
Lipase1
- Dietary restriction has shown promise for disorders such as lysosomal acid lipase deficiency (Wolman disease), as has incorporation of lipid-lowering drugs in the regimen along with sebelipase alpha, a recombinant enzyme replacement therapy. (medscape.com)
Lactase1
- [17] Lactose is normally cleaved in the small intestine by an enzyme called lactase (also known as β-galactosidase). (allergyresearchgroup.com)
Invertase2
- The formula supplies the added support of alpha-galactosidase and beta-glucanase to break down vegetables, beans and grains, and enzymes such as glucoamylase and invertase to assist with the digestion of sugars. (allstarhealth.com)
- I am now taking Muccostop with xylanase , invertase, hemicellulase, mucolase, alpha galactosidase etc between meals on an empty stomach. (curezone.com)
Beta-glucanase1
- Dezember 2013 zur Zulassung einer Zubereitung von alpha-Galactosidase aus Saccharomyces cerevisiae (CBS 615.94) und Endo-1,4-beta-Glucanase aus Aspergillus niger (CBS 120604) als Zusatzstoff in Futtermitteln für. (efta.int)
Enzymes5
- The enzymes responsible for hydrolyzing galactosidic linkages are termed as galactosidases. (springeropen.com)
- Humans have two enzymes with α-galactosidase activity and an acidic pH optimum, α-N-acetylgalactosaminidase (α-NAGA) (previously called α-galactosidase B) and α-galactosidase A (α-GAL). (biomedcentral.com)
- Digestive enzymes like those found in Digesta Alpha improve gastrointestinal (GI) functioning and better health. (digestahealth.com)
- One of the most popular carbohydrase enzymes is alpha-galactosidase. (deerland.com)
- PMID- 214397 TI - Influence of alpha-naphthylthiourea on blood glucose & some enzymes in albino rats. (nih.gov)
1.222
- The α-galactosidases (α-D-galactosidegalactohydrolase, EC 3.2.1.22) are carbohydrases that catalyze the hydrolysis of α-galactosidic linkages in galacto-oligosaccharides such as raffinose, melibiose, stachyose, verbascose and ajugose, polysaccharide-like galactomannans, and glycoconjugates such as glycoproteins and glycolipids. (springeropen.com)
- Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). (nih.gov)
Gene10
- in the second tier, patients with the lowest alpha-galactosidase levels were further subjected to mutation analysis of the GLA gene. (medscape.com)
- Indicated for adults in whom a diagnosis of Fabry disease has been confirmed and who possess an amenable galactosidase-α gene ( GLA ) variant (as determined through in vitro assay data). (medscape.com)
- Multifocal white matter lesions associated with the D313Y mutation of the α-galactosidase A gene. (scielo.br)
- The GLA gene provides instructions for making an enzyme called alpha-galactosidase A. This enzyme is active in lysosomes, which are structures that act as recycling centers within cells. (nih.gov)
- The most common type of mutation changes a single protein building block (amino acid) in alpha-galactosidase A. Other mutations delete part of the GLA gene, insert extra genetic material into the gene, or insert a premature stop signal in the gene's instructions for making alpha-galactosidase A. Alterations in the GLA gene produce an abnormal version of the enzyme that is unable to break down globotriaosylceramide effectively. (nih.gov)
- This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. (antikoerper-online.de)
- The cause of FD is a variety of mutations in the GLA gene on the X chromosome (Xq22.1), resulting in a deficiency of the lysosomal enzyme alpha-galactosidase A (AGAL). (skintherapyletter.com)
- A mutation in a gene called alpha-galactosidase A , or GLA, leads to deficient levels of the alpha-galactosidase A enzyme, which allows the fat to accumulate. (the-scientist.com)
- Fabry disease is an X-linked storage disease due to mutations in the GLA gene encoding the lysosomal enzyme α -galactosidase A, leading to the accumulation of enzyme substrates, namely, globotriaosylceramide (Gb3), lyso-globotriaosylceramide (lyso-Gb3), and galabiosylceramide [ 1 ]. (hindawi.com)
- Fabry disease is caused by mutations in the GLA gene, which result in absent or markedly reduced activity of the alpha-galactosidase A (Gal A) enzyme. (fabrydiseasenews.com)
Lactose3
- 6. The medium as claimed in claim 5, wherein said substrate for the metabolic activity is specific for E. coli and is selected from a substrate for beta-glucuronidase, beta-galactosidase or alpha-galactosidase, for acidification of lactose, or for tryptophanase, beta-ribosidase, phosphatase, L-alanine aminopeptidase or L-leucine aminopeptidase. (freepatentsonline.com)
- Beta-galactosidase -- The enzyme that splits lactose into glucose and galactose. (nih.gov)
- The steady state values, attained upon simulation of model equations, for Allolactose (A), mRNA (M), beta-galactosidase (B), Lactose (L), and Permease (P) match with those predicted by the paper. (nih.gov)
Deficiency of alpha-galactosidase2
- Agalsidase beta is used in the treatment of Fabry disease (a deficiency of alpha-galactosidase A enzyme). (uofmhealth.org)
- It generates a dysfunction of glycosphingolipid metabolism due to an enzymatic deficiency of alpha-galactosidase activity, resulting in glycosphingolipid deposits in all areas of the body. (unibe.ch)
Blend1
- Digestive Defense Blend, Alpha Galactosidase. (spicefruit.com)
Fabry Disease10
- Anderson-Fabry disease (AFD) is an X-linked condition originating from a deficiency in alpha-galactosidase, a lysosomal enzyme. (medscape.com)
- Anderson-Fabry disease (AFD) originates from a deficiency in alpha-galactosidase A (AGALA), a lysosomal enzyme. (medscape.com)
- Mutations that eliminate the activity of the alpha-galactosidase A enzyme lead to the severe, classic form of Fabry disease, which typically begins in childhood. (nih.gov)
- The molecular defect leading to Fabry disease: structure of human alpha-galactosidase. (nih.gov)
- Matsuzawa F, Aikawa S, Doi H, Okumiya T, Sakuraba H. Fabry disease: correlation between structural changes in alpha-galactosidase, and clinical and biochemical phenotypes. (nih.gov)
- Background Fabry disease (α-galactosidase deficiency) is an X-linked genetic disease caused by a variety of pathogenic GLA variants. (bmj.com)
- In males, alpha-galactosidase assays are used to diagnose Fabry disease. (medscape.com)
- Because levels of Gb 3 and alpha-galactosidase A may be normal in female (heterozygote) patients, genetic and/or molecular diagnosis is necessary to confirm Fabry disease in female patients with clinical features. (medscape.com)
- Fenetically engineered, glycosylated human alpha-galactosidase isoenzyme A used in treatment of Fabry disease. (nih.gov)
- Enzyme replacement in Fabry disease: treatment of cultured skin fibroblasts with a purified alpha-galactosidase from ficin. (utmb.edu)
Protein1
- Among his feats, Subramaniam was able to visualize the beta-galactosidase protein with single-particle cryo-EM at an average spatial resolution of 0.22 nanometers-a level of near-atomic detail once considered the exclusive purview of X-ray crystallography. (nih.gov)
Beano1
- Beano contains alpha galactosidase, an enzyme that is taken before meals, to break down complex carbohydrates in food to reduce gas production. (gas-x.com)
Escherichia3
- Analysis of melibiose mutants deficient in alpha-galactosidase and thiomethylgalactoside permease II in Escherichia coli K-12. (wikidata.org)
- Regulation of enzymatic activity in the intact cell: the beta-D-galactosidase of Escherichia coli. (wikidata.org)
- Galactose stimulation of beta-galactosidase induction in galactokinaseless mutants of Escherichia coli. (wikidata.org)
Trichoderma2
- Nutrizyme®AG2, alfa-galactosidase er fremstillet af Trichoderma spp. (enzymes.bio)
- galactosidase A in the filamentous fungus Trichoderma reesei. (antikoerper-online.de)
Catalytic1
- The monomer folds into a catalytic (alpha/beta)(8) barrel and a C-terminal beta-sandwich domain with unassigned function. (nih.gov)
Lipoic Acid2
- Please note that 25% off applies to single bottles only and cannot be used in conjunction with multipack offers Antioxidant and Free Radical Neutralizer Alpha-Lipoic Acid™ is a 'network' antioxidant that has been studied for its potential abilities in the repair of oxidative damage, regeneration of other antioxidants, anti-inflammatory properties and. (goodhealthusa.com)
- It also contains the protective nutrients alpha lipoic acid, NAC, inositol and choline to complement the neural, cognitive, cardiovascular, liver and skin health properties of this diverse profile. (healthywithjodi.com)
Amenable1
- Migalastat is a pharmacological chaperone that is designed to selectively and reversibly bind with high affinity to the active sites of certain mutant forms of alpha-Gal A, the genotypes of which are referred to as amenable mutations. (nih.gov)
Starches1
- Galactosidase breaks down these starches and foods that are difficult to digest. (dinosaurnutritionusa.com)
Digestion2
- An essential enzyme for healthy digestion, Alpha Galactosidase helps to breakdown carbohydrates commonly found in plants to help prevent bloating and discomfort. (spicefruit.com)
- Alpha Galactosidase is an enzyme that increases the breakdown of galactoside in order to improve digestion and reduces bloating, discomfort, and flatulence caused by gas. (dinosaurnutritionusa.com)
Enzyme replace1
- Neuropathic pain agents, enzyme replacement therapy (ERT), or α-galactosidase A (α-Gal A) stabilizers may be indicated. (medscape.com)
Deficient1
- GLA -coding region mutations that cause deficient α-galactosidase A (αGLA) enzyme activity may result in this disease ( 1 1. (scielo.br)
Carbohydrates3
- Alpha-galactosidase help digest carbohydrates. (naturalfoodseries.com)
- This dual-action formula also includes Alpha Galactosidase to help your body break down carbohydrates to help prevent bloating and discomfort. (spicefruit.com)
- Unlike many other gas medicines, Alpha Galactosidase can help prevent gas before it starts by breaking down the complex carbohydrates found in gassy foods into simpler, more easily digestible sugars. (drguberman.com)
Aspergillus1
- 65 BGU e *alpha-Galactosidase (Aspergillus niger) (min). (lambertvetsupply.com)
Galactose3
- Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. (uniprot.org)
- α-galactosidases and β-galactosidases depending on the configuration of the anomeric carbon atom of galactose in the substrate molecule on which they act. (springeropen.com)
- Alpha-Galactosidase ist eine Exoglycosidase, die α-D-Galactose spaltet und zahlreiche Anwendungen in der Medizin-, Biotechnologie-, Lebensmittel- und Pharmaindustrie hat. (eapb.org)
Globotriaosylceramide2
- Binding to and stabilizing endogenous α-galactosidase A (α-Gal A), migalastat chaperones the enzyme from the endoplasmic reticulum to lysosomes, where α-Gal A can degrade the accumulated glycolipid (globotriaosylceramide [GL-3]) and globotriaosylsphingosine (lyso-Gb3). (medscape.com)
- Alpha-galactosidase A breaks down a molecule called globotriaosylceramide, which consists of three sugars attached to a fatty substance. (nih.gov)
Saccharomyces1
- The crystal structures of tetrameric Saccharomyces cerevisiae alpha-galactosidase and its complexes with the substrates melibiose and raffinose have been determined to 1.95, 2.40, and 2.70 A resolution. (nih.gov)
Characterization1
- High-yield production and biochemical characterization of α-galactosidase produced from locally isolated Penicillium sp. (springeropen.com)
Lack the enzyme2
- The thing about raffinose is that it passes through the small intestine undigested because we lack the enzyme alpha-galactosidase. (diabeticmediterraneandiet.com)
- Humans lack the enzyme to break down GOS, alpha-galactosidase. (fanaticcook.com)
Substrate2
- K-m and V-max using p-nitrophenyl-alpha-D-galactopyranoside (PNPG) as substrate were 1.07 mM and 0.01 U/mg, respectively. (ege.edu.tr)
- Coformulation of a Novel Human α-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice. (antikoerper-online.de)
Active ingredient1
- The active ingredient is the enzyme alpha-galactosidase. (diabeticmediterraneandiet.com)
Breakdown1
- Chapter 4, the Alpha Galactosidase breakdown data are shown at the regional level, to show the sales, revenue, and growth by regions. (pharmiweb.com)
Bacteria1
- α-Galactosidase from bacteria, especially probiotic bacteria like bifido and lactic acid bacteria are used as "live cultures" in fermented soymilk for removal of RFOs or in the production of α-galactosidases (Farzadi et al. (springeropen.com)
Units1
- Alpha-galactosidases catalyze the hydrolysis of terminal alpha-1,6-galactosyl units from galacto-oligosaccharides and polymeric galactomannans. (nih.gov)
Extract1
- The highest product yield of α-galactosidase was obtained with media containing yeast extract (6.672 U/ml) as organic nitrogen and ammonium nitrate (6.325 U/ml) and as inorganic nitrogen source with media pH 5.5, and the time course of enzyme production was at the 5th day of fermentation, respectively. (springeropen.com)
Galactomannans1
- 1997 ). α-Galactosidase is also used to improve the gelling properties of galactomannans to be used as food thickeners (Chen and Mustapha 2012 ). (springeropen.com)