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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 ...
Alpha-galactosidase (EC 3.2.1.22) (melibiase) [8] catalyzes the hydrolysis of melibiose into galactose and glucose. In man, the deficiency of this enzyme is the cause of Fabry's disease (X-linked sphingolipidosis). Alpha-galactosidase is present in a variety of organisms.
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 ...
Deficient activity of lysosomal alpha-galactosidase results in progressive accumulation of globotriaosylceramide (GL-3) within lysosomes, that is believed to trigger a cascade of cellular events. [15] The demonstration of marked alpha-galactosidase deficiency is the conclusive method for the diagnosis in homozygous males.
Rose Brystowski, 68, had a choice to make. Others might have found it difficult. She found it easy. Brystowski, of Oak Park, Michigan, wasn't about to let her genetics forfeit her future. Doctors ...
Melibiose is a reducing disaccharide formed by an α-1,6 linkage between galactose and glucose (D-Gal-(α1→6)-D-Glc). [1] [2] It differs from lactose in the chirality of the carbon where the galactose ring is closed and that the galactose is linked to a different point on the glucose moiety.
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