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Article

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 15 Jun 2002

Garden Gwenn A, Libby Randell T, Fu Ying-Hui, Kinoshita Yoshito, Huang Jing, Possin Daniel E, Smith Annette C, Martinez Refugio A, Fine Gabriel C, Grote Sara K, Ware Carol B, Einum David D, Morrison Richard S, Ptacek Louis J, Sopher Bryce L, La Spada Albert R

Abstract excerpt

Spinocerebellar ataxia (SCA) type 7 is an inherited neurodegenerative disorder caused by expansion of a polyglutamine tract within the ataxin-7 protein. To determine the molecular basis of polyglutamine neurotoxicity in this and other related disorders, we produced SCA7 transgenic mice that express ataxin-7 with 24 or 92 glutamines in all neurons of the CNS, except for Purkinje cells. Transgenic mice expressing...

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PMID: 12077187
PMCID: PMC6757746

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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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