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Article

Novel BAC Mouse Model of Huntington's Disease with 225 CAG Repeats Exhibits an Early Widespread and Stable Degenerative Phenotype.

Journal of Huntington's disease - 1 Jan 2015

Wegrzynowicz Michal, Bichell Terry Jo, Soares Barbara D, Loth Meredith K, McGlothan Jennifer S, Mori Susumu, Alikhan Fatima S, Hua Kegang, Coughlin Jennifer M, Holt Hunter K, Jetter Christopher S, Pomper Martin G, Osmand Alexander P, Guilarte Tomás R, Bowman Aaron B

Abstract excerpt

BACKGROUND: Unusually large CAG repeat expansions (>60) in exon one of Huntingtin (HTT) are invariably associated with a juvenile-onset form of Huntington's disease (HD), characterized by a more extensive and rapidly progressing neuropathology than the more prevalent adult-onset form. However, existing mouse models of HD that express the full-length Htt gene with CAG repeat lengths associated with juvenile HD...

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Identifiers

PMID: 26333255
PMCID: PMC4657874

Genes

HTT

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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