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Article

Glial degeneration with oxidative damage drives neuronal demise in MPSII disease.

Cell death & disease - 11 Aug 2016

Zalfa Cristina, Verpelli Chiara, D'Avanzo Francesca, Tomanin Rosella, Vicidomini Cinzia, Cajola Laura, Manara Renzo, Sala Carlo, Scarpa Maurizio, Vescovi Angelo Luigi, De Filippis Lidia

Abstract excerpt

Mucopolysaccharidosis type II (MPSII) is a lysosomal storage disorder due to the deficit of the iduronate 2-sulfatase (IDS) enzyme, causing progressive neurodegeneration in patients. Neural stem cells (NSCs) derived from the IDS-ko mouse can recapitulate MPSII pathogenesis in vitro. In differentiating IDS-ko NSCs and in the aging IDS-ko mouse brain, glial degeneration precedes neuronal degeneration. Here we show...

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Identifiers

PMID: 27512952
DOI: 10.1038/cddis.2016.231
PMCID: PMC5108318

Genes

IDS

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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