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Article

Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1.

Neuron - 15 Feb 2023

Coffin Stephanie L, Durham Mark A, Nitschke Larissa, Xhako Eder, Brown Amanda M, Revelli Jean-Pierre, Villavicencio Gonzalez Esmeralda, Lin Tao, Handler Hillary P, Dai Yanwan, Trostle Alexander J, Wan Ying-Wooi, Liu Zhandong, Sillitoe Roy V, Orr Harry T, Zoghbi Huda Y

Abstract excerpt

Spinocerebellar ataxia type 1 (SCA1) is a paradigmatic neurodegenerative disease in that it is caused by a mutation in a broadly expressed protein, ATXN1; however, only select populations of cells degenerate. The interaction of polyglutamine-expanded ATXN1 with the transcriptional repressor CIC drives cerebellar Purkinje cell pathogenesis; however, the importance of this interaction in other vulnerable cells...

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Identifiers

PMID: 36577402
DOI: 10.1016/j.neuron.2022.11.016
PMCID: PMC9957872

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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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