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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.
Most commonly, galactosemia is caused by a mutation in the GALT gene. The GALT gene normally provides instructions for making an enzyme that functions in liver cells to break down the sugar galactose.f both parents are carriers, there is a 25% chance their child will have galactosemia. There are three different types of galactosemia. Each one is caused by a mutation in a different gene. Classic galactosemia is the most common form of this disorder, and the most severe.