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Alpha-galactosidase is an enzyme, which is derived from the fungus Aspergillus niger. Itâs a homodimeric glycoprotein and it hydrolyzes the terminal alpha-galactosyl moieties (in chemistry moiety is defined as a functional group on a molecule) from glycolipids and glycoproteins.
Classic galactosemia occurs when an enzyme called galactose-1-phosphate uridyltransferase (GALT) is missing or not functional. This liver enzyme is responsible for breaking down galactose (a sugar byproduct of lactose found in breast milk, cowâs milk and other dairy foods) into glucose.