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Article

Neuronal-specific impairment of heparan sulfate degradation in Drosophila reveals pathogenic mechanisms for Mucopolysaccharidosis type IIIA.

Experimental neurology - 1 May 2018

Webber Dani L, Choo Amanda, Hewson Laura J, Trim Paul J, Snel Marten F, Hopwood John J, Richards Robert I, Hemsley Kim M, O'Keefe Louise V

Abstract excerpt

Mucopolysaccharidosis type IIIA (MPS IIIA) is a lysosomal storage disorder resulting from the deficit of the N-sulfoglucosamine sulfohydrolase (SGSH) enzyme that leads to accumulation of partially-degraded heparan sulfate. MPS IIIA is characterized by severe neurological symptoms, clinically presenting as Sanfilippo syndrome, for which no effective therapy is available. The lysosomal SGSH enzyme is conserved in...

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Source

Identifiers

PMID: 29408731
DOI: 10.1016/j.expneurol.2018.01.020

Genes

SGSH

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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