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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
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 ...
When the target gene is not found in the vector, the alpha fragment gene would be active, producing the alpha fragment and allowing for B-galactosidase to gain its activity. To trace the activity of B-galactosidase a colorless analog of lactose is used, X-gal. The hydrolysis of X-gal by B-galactosidase produces galactose, a blue colored compound.
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 ...
Melibiose can be broken down into its component saccharides, glucose and galactose, by the enzyme alpha-galactosidase, such as MEL1 from Saccharomyces pastorianus (lager yeast). Melibiose cannot be used by Saccharomyces cerevisiae [ 3 ] (ale yeast), so this is one test to differentiate between the two yeast species.
Migalastat is used for the long-term treatment of Fabry disease in adults and adolescents aged 16 or older with an amenable mutation of the enzyme alpha-galactosidase A (α-GalA). An "amenable" mutation is one that leads to misfolding of the enzyme, but otherwise would not significantly impair its function. [8]
Raffinose can be hydrolyzed to D-galactose and sucrose by the enzyme α-galactosidase (α-GAL), an enzyme synthesized by bacteria found in the large intestine. α-GAL also hydrolyzes other α-galactosides such as stachyose, verbascose, and galactinol, if present. In plants, raffinose plays a significant role in stress responses, particularly ...
Pegunigalsidase alfa, sold under the brand name Elfabrio, is an enzyme replacement therapy for the treatment of Fabry disease. [2] [4] It is a recombinant human α-galactosidase-A. [4] It is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme. [2] The most common side effects are infusion-related reactions, hypersensitivity and ...
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