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Article

Cytoplasmic targeting of mutant poly(A)-binding protein nuclear 1 suppresses protein aggregation and toxicity in oculopharyngeal muscular dystrophy.

Traffic (Copenhagen, Denmark) - 1 Sept 2005

Abu-Baker Aida, Laganiere Simon, Fan Xueping, Laganiere Janet, Brais Bernard, Rouleau Guy A

Abstract excerpt

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by progressive eyelid drooping, swallowing difficulties and proximal limb weakness. The autosomal dominant form of this disease is caused by a polyalanine expansion from 10 to 12-17 residues, located at the N-terminus of the poly(A)-binding protein nuclear 1 (PABPN1). A distinct pathological hallmark of OPMD is the presence of...

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Source

Identifiers

PMID: 16101680
DOI: 10.1111/j.1600-0854.2005.00315.x

Genes

PABPN1

Provenance

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.

Related research

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Cytoplasmic targeting of mutant poly(A)-binding protein nuclear 1 suppresses protein aggregation and toxicity in oculopharyngeal muscular dystrophy.PMID 16101680Read ↗

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hnRNP A1 and A/B Interaction with PABPN1 in Oculopharyngeal Muscular DystrophySemantic proximity Read ↗

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Oligomerization of polyalanine expanded PABPN1 facilitates nuclear protein aggregation that is associated with cell death.Semantic proximity Read ↗

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Oculopharyngeal muscular dystrophy: a late-onset polyalanine disease.Semantic proximity Read ↗

Oculopharyngeal muscular dystrophy (OPMD) associated alanine expansion impairs the function of the nuclear polyadenosine RNA binding protein PABPN1 as revealed by proximity labeling and comparative proteomicsSemantic proximity Read ↗

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