Complement C3c: A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.alpha-Galactosidase: An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids.Fabry Disease: An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.Galactosidases: A family of galactoside hydrolases that hydrolyze compounds with an O-galactosyl linkage. EC 3.2.1.-.beta-Galactosidase: A group of enzymes that catalyzes the hydrolysis of terminal, non-reducing beta-D-galactose residues in beta-galactosides. Deficiency of beta-Galactosidase A1 may cause GANGLIOSIDOSIS, GM1.Cerebrosides: Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)Lactose: A disaccharide of GLUCOSE and GALACTOSE in human and cow milk. It is used in pharmacy for tablets, in medicine as a nutrient, and in industry.Galactose: An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.Acridine Orange: A cationic cytochemical stain specific for cell nuclei, especially DNA. It is used as a supravital stain and in fluorescence cytochemistry. It may cause mutations in microorganisms.alpha-L-Fucosidase: An enzyme that catalyzes the hydrolysis of an alpha L-fucoside to yield an alcohol and L-fucose. Deficiency of this enzyme can cause FUCOSIDOSIS. EC 3.2.1.51.Nigericin: A polyether antibiotic which affects ion transport and ATPase activity in mitochondria. It is produced by Streptomyces hygroscopicus. (From Merck Index, 11th ed)Glycoside HydrolasesGlycosides: Any compound that contains a constituent sugar, in which the hydroxyl group attached to the first carbon is substituted by an alcoholic, phenolic, or other group. They are named specifically for the sugar contained, such as glucoside (glucose), pentoside (pentose), fructoside (fructose), etc. Upon hydrolysis, a sugar and nonsugar component (aglycone) are formed. (From Dorland, 28th ed; From Miall's Dictionary of Chemistry, 5th ed)Cell Aging: The decrease in the cell's ability to proliferate with the passing of time. Each cell is programmed for a certain number of cell divisions and at the end of that time proliferation halts. The cell enters a quiescent state after which it experiences CELL DEATH via the process of APOPTOSIS.Histocytochemistry: Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.Escherichia coli: A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.alpha 1-Antitrypsin: Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Receptors, Adrenergic, alpha: One of the two major pharmacological subdivisions of adrenergic receptors that were originally defined by the relative potencies of various adrenergic compounds. The alpha receptors were initially described as excitatory receptors that post-junctionally stimulate SMOOTH MUSCLE contraction. However, further analysis has revealed a more complex picture involving several alpha receptor subtypes and their involvement in feedback regulation.Hypoxia-Inducible Factor 1, alpha Subunit: Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by OXYGEN availability and is targeted for degradation by VHL TUMOR SUPPRESSOR PROTEIN.alpha7 Nicotinic Acetylcholine Receptor: A member of the NICOTINIC ACETYLCHOLINE RECEPTOR subfamily of the LIGAND-GATED ION CHANNEL family. It consists entirely of pentameric a7 subunits expressed in the CNS, autonomic nervous system, vascular system, lymphocytes and spleen.Integrin alpha3beta1: Cell surface receptor for LAMININ, epiligrin, FIBRONECTINS, entactin, and COLLAGEN. Integrin alpha3beta1 is the major integrin present in EPITHELIAL CELLS, where it plays a role in the assembly of BASEMENT MEMBRANE as well as in cell migration, and may regulate the functions of other integrins. Two alternatively spliced isoforms of the alpha subunit (INTEGRIN ALPHA3), are differentially expressed in different cell types.Integrin alpha4: An integrin alpha subunit that is unique in that it does not contain an I domain, and its proteolytic cleavage site is near the middle of the extracellular portion of the polypeptide rather than close to the membrane as in other integrin alpha subunits.Integrin alpha6: An integrin alpha subunit that primarily associates with INTEGRIN BETA1 or INTEGRIN BETA4 to form laminin-binding heterodimers. Integrin alpha6 has two alternatively spliced isoforms: integrin alpha6A and integrin alpha6B, which differ in their cytoplasmic domains and are regulated in a tissue-specific and developmental stage-specific manner.Integrin alpha5beta1: An integrin found in FIBROBLASTS; PLATELETS; MONOCYTES, and LYMPHOCYTES. Integrin alpha5beta1 is the classical receptor for FIBRONECTIN, but it also functions as a receptor for LAMININ and several other EXTRACELLULAR MATRIX PROTEINS.Integrin alpha4beta1: Integrin alpha4beta1 is a FIBRONECTIN and VCAM-1 receptor present on LYMPHOCYTES; MONOCYTES; EOSINOPHILS; NK CELLS and thymocytes. It is involved in both cell-cell and cell- EXTRACELLULAR MATRIX adhesion and plays a role in INFLAMMATION, hematopoietic cell homing and immune function, and has been implicated in skeletal MYOGENESIS; NEURAL CREST migration and proliferation, lymphocyte maturation and morphogenesis of the PLACENTA and HEART.Interleukin-1alpha: An interleukin-1 subtype that occurs as a membrane-bound pro-protein form that is cleaved by proteases to form a secreted mature form. Unlike INTERLEUKIN-1BETA both membrane-bound and secreted forms of interleukin-1alpha are biologically active.Integrin alpha2beta1: An integrin found on fibroblasts, platelets, endothelial and epithelial cells, and lymphocytes where it functions as a receptor for COLLAGEN and LAMININ. Although originally referred to as the collagen receptor, it is one of several receptors for collagen. Ligand binding to integrin alpha2beta1 triggers a cascade of intracellular signaling, including activation of p38 MAP kinase.Receptors, Adrenergic, alpha-1: A subclass of alpha-adrenergic receptors that mediate contraction of SMOOTH MUSCLE in a variety of tissues such as ARTERIOLES; VEINS; and the UTERUS. They are usually found on postsynaptic membranes and signal through GQ-G11 G-PROTEINS.Diphtheria-Tetanus-Pertussis Vaccine: A vaccine consisting of DIPHTHERIA TOXOID; TETANUS TOXOID; and whole-cell PERTUSSIS VACCINE. The vaccine protects against diphtheria, tetanus, and whooping cough.Rotavirus Vaccines: Vaccines or candidate vaccines used to prevent infection with ROTAVIRUS.Immunization Schedule: Schedule giving optimum times usually for primary and/or secondary immunization.Vaccines, Combined: Two or more vaccines in a single dosage form.Diphtheria-Tetanus-acellular Pertussis Vaccines: 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.PubMed: 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.Periodicals as Topic: A publication issued at stated, more or less regular, intervals.BooksPublishing: "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.MEDLINE: 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).Databases, Protein: Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.Internet: 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.User-Computer Interface: The portion of an interactive computer program that issues messages to and receives commands from a user.Crystallography, X-Ray: The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Models, Molecular: Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.Software: Sequential operating programs and data which instruct the functioning of a digital computer.Antibodies: 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).Angiokeratoma: A vascular, horny neoplasm of the skin characterized by TELANGIECTASIS and secondary epithelial changes including acanthosis and hyperkeratosis.Pathology, Molecular: A subspecialty of pathology concerned with the molecular basis (e.g., mutations) of various diseases.Pseudotsuga: 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.Trihexosylceramides: 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).Eye Manifestations: 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)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". J. Biol. Chem. 273 (42): ...
"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 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 ...
... 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 ... van Beeumen J, Kroos MA, Oostra BA, Hermans MM, Reuser AJ (1991). "Human lysosomal alpha-glucosidase. Characterization of the ... 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". Eur. J. Biochem. 202 (2 ...
It contains the enzymes alpha-galactosidase (α-GAL) and invertase. It was introduced as a liquid, but that has been ... "The effect of oral alpha-galactosidase on intestinal gas production and gas-related symptoms". Dig. Dis. Sci. 52 (1): 78-83. ... A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". J Fam Pract. 39 (5): ...
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. ...
Fabrazyme (agalsidase beta, or Alpha-galactosidase) was licensed to the Genzyme Corporation. It is an enzyme replacement ... A deficiency of the enzyme alpha galactosidase A (a-GAL A, encoded by GLA) due to mutation causes a glycolipid known as ... to measure the level of alpha-galactosidase activity. An enzyme assay is not reliable for the diagnosis of disease in females ... The pharmaceutical company Shire manufactures agalsidase alpha (which differs in the structure of its oligosaccharide side ...
doi:10.1007/s12257-010-0069-0. Dey PM1, Patel S, Brownleader MD (June 1993). "Induction of alpha-galactosidase in Penicillium ...
... 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 ... "The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines". ... of Fabry disease in adults and adolescents aged 16 or older with an amenable mutation of the enzyme alpha-galactosidase A (α- ... ISBN 0-470-03391-6. Miyake, Y; Ebata, M (1988). "The structures of a β-galactosidase inhibitor, Galactostatin, and its ...
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- ...
... 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 ...
Supplements of the enzyme supplement alpha-galactosidase may reduce symptoms (if brands containing other FODMAPs are avoided). ...
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, ...
Other genetically modified pigs have had alpha galactosidase transferase knocked out and fortified with hCD46 and the hTM ... Human-alpha-1-antitrypsin, which has been tested in sheep and is used in treating humans with this deficiency and transgenic ... 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 (3): 293-303 ...
1995). "Sixty-nine kilobases of contiguous human genomic sequence containing the alpha-galactosidase A and Bruton's tyrosine ...
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): ...
... plant cell culture expressed and a chemically modified version of the recombinant alpha-Galactosidase-A protein. Protein sub- ...
Alpha-galactosidase, an enzyme that breaks down certain complex sugars, is a component of Beano and other products that ...
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 ...
The enzyme alpha-galactosidase now is allowed to be produced in moss bioreactors by the German Federal Institute for Drugs and ...
Some species of mold produce alpha-galactosidase, an anti-oligosaccharide enzyme, which humans can take to facilitate digestion ...
Beta-galactosidase. *Hexosaminidase. *mannosidase *alpha-Mannosidase. *beta-mannosidase. *Aspartylglucosaminidase. *Fucosidase ...
Beta-galactosidase Migalastat, a drug targeting alpha-galactosidase Classification of α-galactosidases (according to CAZy) ... Dean KJ, Sweeley CC (1979). "Studies on human liver alpha-galactosidases. I. Purification of alpha-galactosidase A and its ... Alpha-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and ... Two recombinant forms of alpha-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN). This enzyme is a ...
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. ...
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 ...
Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Crystal structure of alpha-galactosidase A at pH 4.5 complexed with 1-deoxygalactonijirimycin. *DOI: 10.2210/pdb3GXT/pdb ... including the first structure of alpha-Gal A with DGJ. Both GCase and alpha-Gal A are more stable at lysosomal pH with and ...
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 ...
Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Alpha-galactosidase A. A, B. 398. Homo sapiens. Mutation(s): 0 Gene Names: GLA. EC: 3.2.1.22 (PDB Primary Data), 3.2.1.47 ( ... Crystal structure of acid-alpha-galactosidase A complexed with galactose at pH 4.5. *DOI: 10.2210/pdb3GXP/pdb ...
... 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, ...
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 ...
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 ...
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 ...
FabryCatalysesLysosomalAgalsidaseLackHumanBeta-galactosidaseFabry Disease1.22Induced alpha-galactosidase activityGalactoseProteinLysosomal enzymeGaluAspergillusGalactohydrolaseHydrolyzesGlobotriaosylceramideMigalastatBeanoHydrolaseEnzymesCharacterizationAntibodyFabry's DiseaseResiduesHeparan sulfateClinicalKinetic propertiesProduced by recombinantHydrolyzeEndothelial cellsAssay1970Recombinant humanContaining lactoseAmino acidFragmentGuarOligosaccharideTerminal
- Fabry disease is caused by a mutation of the alpha-galactosidase A gene (GLA) mapped to the long arm of the X chromosome . (dermnetnz.org)
- Pathogenetic GLA variants cause alpha-galactosidase A (α-Gal A) enzyme deficiency leading to the X-linked lysosomal storage disorder Fabry disease (FD). (pubfacts.com)
- 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. (unipi.it)
- Fabry disease (also called alpha-galactosidase-A deficiency) is caused by the lack of or faulty enzyme needed to metabolize lipids, fat-like substances that include oils, waxes, and fatty acids. (brainandlife.org)
- The enzyme alpha-galactosidase A catalyses the separation ( cleavage ) of the terminal galactose from globotriaosylceramide (Gb3). (dermnetnz.org)
- Alpha-galactosidase (αGal) is a lysosomal enzyme that hydrolyses the alphagalactosyl moiety from glycosphingo-lipids. (pubfacts.com)
- Agalsidase beta is a man-made form of the naturally-occurring alpha-galactosidase A enzyme. (wellspan.org)
- Lack of the alpha-galactosidase A enzyme leads to accumulation of Gb3 in various tissues, leading to cell death. (dermnetnz.org)
- Antibody Epitope of human α-Galactosidase A revealed by affinity-mass spectrometry: A basis for reversing immunoreactivity in enzyme replacement therapy of Fabry's Disease. (pubfacts.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)
- For details on β-galactosidase see Molecular Playground/Beta-galactosidase . (proteopedia.org)
- Three-dimensional structure of beta-galactosidase from E. coli. (proteopedia.org)
- 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)
- the beta-galactanase, which appears around day 7 of development, was separated from alpha- and beta-galactosidase which emerge at day 1 and 4 after oviposition, respectively. (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 and sphingolipidosis that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. (wikipedia.org)
- Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A". Proceedings of the National Academy of Sciences of the United States of America. (wikipedia.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)
- 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 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)
- 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)
- Desnick RJ, Ioannou YA, Eng CM. a-Galactosidase A deficiency: Fabry disease. (wikipedia.org)
- Anderson-Fabry disease (AFD) is an X-linked disease of lysosomal metabolism resulting in attenuated activity or absence of the enzyme alpha-galactosidase A (a-Gal A). Impaired glycosphingolipid metabolism leads to systemic lysosomal globotriaosylceramide accumulation with multiorgan systemic involvement and complex clinical presentation: acroparesthesias, angiokeratoma, hypohidrosis, corneal and lenticular opacities, gastrointestinal and endocrine abnormalities, renal impairment, and neural and cardiovascular disease [ 1 , 2 ]. (hindawi.com)
- 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)
- 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)
- 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)
- We show that the conformations of the active site loops in GCase are sensitive to ligand binding but not pH, whereas analogous galactose- or DGJ-dependent conformational changes in alpha-Gal A are not seen. (rcsb.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 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)
- 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)
- It is formed by the alpha linkage of galactose to lactosylceramide catalyzed by A4GALT . (wikipedia.org)
- 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)
- 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)
- Alpha-galactosidase 260 GalU. (hqhair.com)
- Alpha-Amylase 62.5mg (5000 DU), Lactase 37.5mg (3000 ALU), Alpha-Galactosidase 29.9mg (260 Galu), Protease 25mg (12500 HUT), Cellulase 11.5mg (400 CU), Lipase 8.6mg (150 LU), Stem Bromelain 1.7mg (50000 PU). (hqhair.com)
- High purity α-Galactosidase ( Aspergillus niger ) for use in research, biochemical enzyme assays and in vitro diagnostic analysis. (megazyme.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)
- 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)
- Hydrolyzes a variety of simple alpha-D-galactoside as well as more complex molecules such as oligosaccharides and polysaccharides. (mybiosource.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)
- α-Galactosidase (Agal) hydrolyzes the terminal α-galactosyl moiety from glycoproteins and glycolipids . (proteopedia.org)
- β-Galactosidase (Bgal) hydrolyzes β-galactosides into monosaccharides . (proteopedia.org)
- It is metabolized by alpha-galactosidase , which hydrolyzes the terminal alpha linkage. (wikipedia.org)
- Alpha-galactosidase A breaks down a molecule called globotriaosylceramide, which consists of three sugars attached to a fatty substance. (medlineplus.gov)
- Fabry's disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme α-galactosidase A, which results in progressive intracellular accumulation of globotriaosylceramide (Gb3) in various organs including the heart. (nii.ac.jp)
- Defects in the enzyme alpha-galactosidase lead to the buildup of globotriaosylceramide, causing Fabry's disease . (wikipedia.org)
- These disturbances are caused by globotriaosylceramide (GL-3) accumulation in the heart resulting from lysosomal α-galactosidase A deficiency. (ahajournals.org)
- The genetic deficiency of α-galactosidase A in Fabry patients leads to the histopathological accumulation of globotriaosylceramide (GL-3) within the lysosomes of multiple cell types throughout the body, particularly in the kidney, skin, and heart. (ahajournals.org)
- The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabry's. (wikipedia.org)
- Alpha-galactosidase is an active ingredient in Beano, CVS BeanAid, Enzymedica's BeanAssist. (wikipedia.org)
- Try Beano (alpha-galactosidase). (wikihow.com)
- Beano and other supplements containing alpha-galactosidase are available in many pharmacies and health food stores. (wikihow.com)
- Alpha-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. (wikipedia.org)
- 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)
- 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)
- Alpha-amylases are digestive enzymes which hydrolyze glycosidic bonds in starch to glucose, maltose, malt triose and dextrin. (alpspure.co.in)
- 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)
- The characterization and properties of a beta-galactanase and alpha- and beta-galactosidases as well as heparan sulfate and chondroitin sulfate degrading enzymes which appear during the 15 days of the embryonic development of the mollusc Pomacea sp. (unifesp.br)
- 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)
- 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)
- 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)
- This paper reports on the effects of both reducing and nonreducing transgalactooligosaccharides (TOS) comprising 2 to 8 residues on the growth of Bifidobacterium adolescentis DSM 20083 and on the production of a novel β-galactosidase (β-Gal II). (asm.org)
- An alpha-N-acetylglucosaminidase and a beta-glucuronidase which act upon the N-acetylated fragments formed from heparan sulfate emerge around day 4 of development. (unifesp.br)
- 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)
- Conclusions- The findings suggest that long-term treatment with recombinant human α-galactosidase A may halt the progression of vascular pathology and prevent the clinical manifestations of atherosclerotic disease. (ahajournals.org)
- Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells. (semanticscholar.org)
- Human a -galactosidase A produced by recombinant DNA technology in cultured human cells. (drugfuture.com)
- 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. (allcosmeticsource.com)
- Methods and Results- We analyzed pretreatment and posttreatment endomyocardial biopsies from 58 Fabry patients enrolled in a 5-month, phase 3, double-blind, randomized, placebo-controlled trial, followed by a 54-month open-label extension study of recombinant human α-galactosidase A. Baseline evaluations revealed GL-3 deposits in interstitial capillary endothelial cells and large, laminated inclusions within cardiomyocytes. (ahajournals.org)
- Results from this assay do not reflect carrier status because of individual variation of alpha-galactosidase enzyme levels. (testcatalog.org)
- Identification as an a -galactosidase: J. A. Kint, Science 167, 1268 (1970). (drugfuture.com)
- We have previously reported the results of enzyme replacement therapy with recombinant human α-galactosidase A (r-hαGalA) on the histological clearance of GL-3 from both renal 1 and dermatologic biopsies 2 obtained during a phase 3 trial. (ahajournals.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)
- 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)
- 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)
- 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)
- Lucerne and guar contained two α-galactosidase activities, carob three and soybean four. (megazyme.com)
- Agalsidase alpha and beta differ in the structures of their oligosaccharide side chains. (wikipedia.org)