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α-Galactosidase ( EC 3.2.1.22, α-GAL, α-GAL A; systematic name α-D-galactoside galactohydrolase) is a glycoside hydrolase enzyme that catalyses the following reaction: [1] Hydrolysis of terminal, non-reducing α- D -galactose residues in α- D -galactosides, including galactose oligosaccharides, galactomannans and galactolipids
Galactosidases are enzymes (glycoside hydrolases) that catalyze the hydrolysis of galactosides into monosaccharides.. Galactosides can be classified as either alpha or beta. If the galactoside is classified as an alpha-galactoside, the enzyme is called alpha-galactosidase, and is responsible for catalyzing the hydrolysis of substrates that contain α-galactosidic residues, such as ...
2717 11605 Ensembl ENSG00000102393 ENSMUSG00000031266 UniProt P06280 P51569 RefSeq (mRNA) NM_000169 NM_013463 RefSeq (protein) NP_000160 NP_038491 Location (UCSC) Chr X: 101.39 – 101.41 Mb Chr X: 133.49 – 133.5 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Galactosidase alpha is an enzyme that in humans is encoded by the GLA gene. Two recombinant forms of human α-galactosidase ...
Alpha-N-acetylgalactosaminidase (EC 3.2.1.49) [10] catalyzes the hydrolysis of terminal non-reducing N-acetyl-D-galactosamine residues in N-acetyl-alpha-D- galactosaminides. In man, the deficiency of this enzyme is the cause of Schindler and Kanzaki diseases. The sequence of this enzyme is highly related to that of the eukaryotic alpha ...
A person who inherits this gene does not have enough of a functioning enzyme known as alpha-galactosidase A. The lack of alpha-galactosidase leads to Fabry disease. A deficiency of alpha galactosidase A (a-GAL A, encoded by GLA) due to mutation causes a glycolipid known as globotriaosylceramide (abbreviated as Gb3, GL-3, or ceramide trihexoside ...
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Raffinose synthase EC 2.4.1.82 is the key enzyme that channels sucrose into the raffinose oligosaccharide pathway. [11] Glycoside hydrolase family 36 also includes enzymes with α-N-acetylgalactosaminidase EC 3.2.1.49 and stachyose synthase EC 2.4.1.67 activities. Glycoside hydrolase family 36 can be subdivided into 11 families, GH36A to GH36K ...
Globotriaosylceramide is a globoside. [1] It is also known as CD77, Gb3, GL3, and ceramide trihexoside. [2] It is one of the few clusters of differentiation that is not a protein. It is formed by the alpha linkage of galactose to lactosylceramide catalyzed by A4GALT. It is metabolized by alpha-galactosidase, which hydrolyzes the terminal alpha ...