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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.
A missing enzyme needed to break down galactose in a person's body causes galactosemia. This disease is an inherited genetic mutation called an autosomal recessive condition.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.