4XWH466927419ENSG00000108784ENSMUSG00000001751P54802O88325NM_000263NM_013792NP_000254NP_038820N-acetylglucosaminidase, alpha is a protein that in humans is encoded by the NAGLU gene.

[5] This gene encodes an enzyme that degrades heparan sulfate by hydrolysis of terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides.

Defects in this gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B.

This disease is characterized by the lysosomal accumulation and urinary excretion of heparan sulfate.

[5] This article incorporates text from the United States National Library of Medicine, which is in the public domain.