Journal Article

Neurological abnormalities in a knock-in mouse model of Huntington’s disease

Chin-Hsing Lin, Sara Tallaksen-Greene, Wei-Ming Chien, Jamie A. Cearley, Walker S. Jackson, Andrew B. Crouse, Songrong Ren, Xiao-Jiang Li, Roger L. Albin and Peter J. Detloff

in Human Molecular Genetics

Volume 10, issue 2, pages 137-144
Published in print January 2001 | ISSN: 0964-6906
Published online January 2001 | e-ISSN: 1460-2083 | DOI:
Neurological abnormalities in a knock-in mouse model of Huntington’s disease

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Mice representing precise genetic replicas of Huntington’s disease (HD) were made using gene targeting to replace the short CAG repeat of the mouse Huntington’s disease gene homolog (Hdh) with CAG repeats within the length range found to cause HD in humans. Mice with alleles of 150 units in length exhibit late-onset behavioral and neuroanatomic abnormalities consistent with HD. These symptoms include a motor task deficit, gait abnormalities, reactive gliosis and the formation of neuronal intranuclear inclusions predominating in the striatum. This model differs from previously described Hdh knock-ins by its method of construction, longer repeat length and more severe phenotype. To our knowledge, this is the first knock-in mouse model of HD to show increased glial fibrillary acidic protein immunoreactivity in the striatum, suggesting that these mice have neuronal injury similar to that found early in the course of HD. These mice will serve as useful reagents in experiments designed to reveal the molecular nature of neuronal dysfunction underlying HD.

Journal Article.  6387 words.  Illustrated.

Subjects: Genetics and Genomics

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