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Article

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice.

Neurobiology of disease - 1 Mar 2009

Morton A Jennifer, Glynn Dervila, Leavens Wendy, Zheng Zhiguang, Faull Richard L M, Skepper Jeremy N, Wight James M

Abstract excerpt

Huntington's disease (HD) is caused by an expanded CAG repeat in the HD gene. The pathological threshold for expansion in HD is around 36 CAG repeats, although 'super-long' expansions are found in brains of HD patients. We examined the effect of varying the CAG repeat length (from 170 to 450) on behavior and neuropathology of R6/2 mice. Unexpectedly, we found that increasing the repeat length delayed onset of...

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Identifiers

PMID: 19130884
DOI: 10.1016/j.nbd.2008.11.015

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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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