N-sulphoglucosamine sulphohydrolase

Definition:

Catalyzes a step in lysosomal heparan sulfate degradation.

References:

[1]. H S Scott, et al. Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome. Nat Genet. 1995 Dec;11(4):465-7. [Content Brief]

[2]. Navdeep S Sidhu, et al. Structure of sulfamidase provides insight into the molecular pathology of mucopolysaccharidosis IIIA. Acta Crystallogr D Biol Crystallogr. 2014 May;70(Pt 5):1321-35. [Content Brief]

[3]. Nicole Muschol, et al. Transport, enzymatic activity, and stability of mutant sulfamidase (SGSH) identified in patients with mucopolysaccharidosis type III A. Hum Mutat. 2004 Jun;23(6):559-66. [Content Brief]

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