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Article

Pathological accumulation of atrophin-1 in dentatorubralpallidoluysian atrophy.

International journal of clinical and experimental pathology - 1 Apr 2011

Suzuki Yasuyo, Yazawa Ikuru

Abstract excerpt

Dentatorubral-pallidoluysian atrophy (DRPLA) is caused by the expansion of polyglutamine (polyQ) in atrophin-1 (ATN1), also known as DRPLA protein. ATN1 is ubiquitously expressed in the central nervous system (CNS), although selective regions of CNS are degenerated in DRPLA, and this selective neuronal damage gives rise to the specific clinical features of DRPLA. Accumulation of mutant ATN1 that carries an...

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Source

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Identifiers

PMID: 21577324
PMCID: PMC3093063

Genes

ATN1

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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