Alpha-N-acetylglucosaminidase (NAGLU) hydrolyses the non-reducing, terminal N-acetyl-D-glucosamine residue from heparan sulfate. The active form of the enzyme (77kDa) is derived from a 82kDa precursor (Weber et al. 1996). Defects in NAGLU cause of mucopolysaccharidosis type IIIB (MPSIIIB, MIM:252920) also known as Sanfilippo syndrome type B (Beesley et al. 2005).
Blanch, L, Weber, B, Hopwood, JJ, Scott, HS, Clements, PR
Beesley, CE, Young, EP, Winchester, BG, Vellodi, A, Jackson, M
alpha-N-acetylglucosaminidase activity of NAGLU(59-743) [lysosomal lumen]
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