Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers Alpha-galactosidase A (GLA) (Bishop et al. 1986) removes the terminal galactose residue from glycolipids or glycoproteins resulting in galactose and an alcohol. An example is the Fabry disease substrate globotriaosylceramide (Gb3Cer) which is hydrolysed to form galactose and lactosylceramide. GLA functions as a homodimer (Garman & Garboczi 2004) and defects in this enzyme lead to Fabry disease (FD) (MIM:301500), a rare X-linked sphingolipidosis disease where glycolipids such as GB3 accumulate in many tissues (Garman & Garboczi 2004, Eng et al. 1993). Multiple mutations in GLA can cause the disease symptoms of Fabry disease (Shabeer et al. 2006). Authored: Jassal, B, 2011-09-21 EC Number: 3.2.1.22 Edited: Jassal, B, 2011-09-21 Pubmed15003450 Pubmed16595074 Pubmed3014515 Pubmed7504405 Reactome Database ID Release 431605736 Reactome, http://www.reactome.org ReactomeREACT_115752 Reviewed: Stephan, R, 2011-10-31