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Article

Soluble expanded PABPN1 promotes cell death in oculopharyngeal muscular dystrophy.

Neurobiology of disease - 1 Jun 2007

Messaed Christiane, Dion Patrick A, Abu-Baker Aida, Rochefort Daniel, Laganiere Janet, Brais Bernard, Rouleau Guy A

Abstract excerpt

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease caused by the expansion of a polyalanine repeat (GCG)(8-13) in exon 1 of the PABPN1 gene. Skeletal muscle fibers nuclei from OPMD patients contain insoluble polyalanine expanded PABPN1 (expPABPN1) nuclear aggregates that sequester different cellular components. Whether these aggregates are pathogenic, or the consequence of a molecular...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 17418585
DOI: 10.1016/j.nbd.2007.02.004

Genes

PABPN1
GCG

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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