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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.
Galactosemia (Galactosaemia) is a rare genetic metabolic disorder that affects an individual's ability to metabolize the sugar galactose properly. Galactosemia is not related to and should not be confused with lactose intolerance. Galactosemia follows an autosomal recessive mode of inheritance that confers a deficiency in an enzyme responsible for adequate galactose degradation. Goppert first described the disease in 1917, with its cause as a defect in galactose metabolism being identified by a group led by Herman Kalckar in 1956.