heparan-α-glucosaminide N-acetyltransferase

hep·a·ran-α-glu·co·sam·i·nide N-ac·e·tyl·trans·fer·ase (hepґə-ran gloo″ko-samґĭ-nīd as″ə-tēl-transґfər-ās) [EC 2.3.1.78] a lysosomal enzyme of the transferase class that catalyzes the acetylation of the amino groups of terminal desulfated glucosamine residues in heparan sulfate chains, a step in the degradation of heparan sulfate. Acetyl coenzyme A is the acetyl donor. Deficiency of the enzyme, an autosomal recessive trait, results in Sanfilippo syndrome, type C. Called also acetyl CoA:α-glucosaminide N-acetyltransferase.