heparan N-sulfatase

heparan N-sulfatase
An enzyme that participates in the stepwise degradation of heparan sulfate; heparan N-sulfatase hydrolyzes the sulfate moiety attached to the amino group of the glucosamine residue of heparan sulfate; a deficiency of this enzyme is associated with mucopolysaccharidose IIIA (Sanfilippo syndrome A).

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hep·a·ran N-sul·fa·tase (hepґə-ran sulґfə-tās) a lysosomal enzyme of the hydrolase class that catalyzes the removal of sulfate from terminal N-sulfated glucosamine residues of heparan sulfate chains. Deficiency of the enzyme, an autosomal recessive trait, causes Sanfilippo syndrome, type A. See also N-sulfoglucosamine sulfohydrolase.

Medical dictionary. 2011.

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