Primary Identifier | IPR035626 | Type | Family |
Short Name | GALNS |
description | Lysosomal galactosamine-6-sulfatase (GALNS) removes sulfate groups from a terminal N-acetylgalactosamine-6-sulfate (or galactose-6-sulfate) in mucopolysaccharides such as keratan sulfate and chondroitin-6-sulfate. Defects in GALNS lead to accumulation of substrates, resulting in the development of the lysosomal storage disease mucopolysaccharidosis IV A []. |