NAGA (N-acetylgalactosaminidase, α), also known as α-galactosidase B or GALB, is a 411 lysosomal protein belonging to the glycosyl hydrolase 27 family that may exist as a homodimer and plays a critical role in glycolipid breakdown. NAGA encodes α-N-acetylgalactosaminidase, a lysosomal enzyme, which cleaves α-N-acetylgalactosaminyl groups from glycoconjugates. Mapping to human chromosome 22q13.2, NAGA defects are the cause of an autosomal recessive disorder with three phenotypes, known as Schindler disease (types I, II and III) or NAGA deficiency (types I, II and III). Characterized by neurologic manifestations that range in severity, Schindler disease type I ...
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