Arylsulfatase A (ASA) - Definition

March 2017


Definition


Arylsulfatase is an enzyme that breaks down specific lipids called sulfate esters. There are two main types of arylsulfatase: A and B. Deficiency in arylsulfatase A (ASA) is a condition known as metachromatic leukodystrophy, while arylsulfatase B deficiency is indicative of Maroteaux-Lamy syndrome or polydystrophic dwarfism. Metachromatic leukodystrophy is a hereditary disease in which sulfate esters accumulate primarily in the nervous system, which causes neurological disorders from the age of 2 affecting walking ability and causing cognitive deterioration followed by death in just a few years.

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