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Article

Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.

PloS one - 30 Nov 2009

Moffitt Hilary, McPhail Graham D, Woodman Ben, Hobbs Carl, Bates Gillian P

Abstract excerpt

BACKGROUND: Huntington's disease (HD) is an inherited progressive neurodegenerative disorder caused by a CAG repeat expansion in the ubiquitously expressed HD gene resulting in an abnormally long polyglutamine repeat in the huntingtin protein. Polyglutamine inclusions are a hallmark of the neuropathology of HD. We have previously shown that inclusion pathology is also present in the peripheral tissues of the R6/2...

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Identifiers

PMID: 19956633
DOI: 10.1371/journal.pone.0008025
PMCID: PMC2778556

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