Α-Galactosidase Explained

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

It catalyzes many catabolic processes, including cleavage of glycoproteins, glycolipids, and polysaccharides.

The enzyme is encoded by the GLA gene.^[2]

Function

This enzyme is a homodimeric glycoprotein that hydrolyses the terminal α-galactosyl moieties from glycolipids and glycoproteins. It predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose.

See also

• β-galactosidase
• Migalastat, a drug targeting α-galactosidase
• Classification of α-galactosidases (according to CAZy)

Notes and References

1. Book: Scriver CR, Sly WS, Childs B, ABeaudet AL, Valle D, Kinzler KW, Vogelstein B . The Metabolic & Molecular Basis of Inherited Disease . McGraw-Hill . 978-0-07-913035-8 . 8th . 15 December 2000.
2. Calhoun DH, Bishop DF, Bernstein HS, Quinn M, Hantzopoulos P, Desnick RJ . Fabry disease: isolation of a cDNA clone encoding human α-galactosidase A . Proceedings of the National Academy of Sciences of the United States of America . 82 . 21 . 7364–8 . November 1985 . 2997789 . 391345 . 10.1073/pnas.82.21.7364 . 1985PNAS...82.7364C . free .