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Overview
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N-Sulphoglucosamine Sulphohydrolase (SGSH) is a member of the sulfatase family that is involved in the degradation of heparin sulfate (HS). SGSH catalyzes N-sulfo-D-glucosamine and H2O to D-glucosamine and sulfate. Unlike the HS specific endosulfatases that remove sulfate from internal GlcNAc residues, SGSH removes sulfate group from the non-reducing end glucosamine residues on HS. SGSH leads to mucopolysaccharidosis type 3A (MPS3A), a recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations.
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