Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

Ioannis Dragatsis, Michael S. Levine, Scott Zeitlin

Research output: Contribution to journalArticle

377 Citations (Scopus)

Abstract

Inactivation of the mouse homologue of the Huntington disease gene (Hdh) results in early embryonic lethality. To investigate the normal function of Hdh in the adult and to evaluate current models for Huntington disease (HD), we have used the Cre/loxP site-specific recombination strategy to inactivate Hdh expression in the forebrain and testis, resulting in a progressive degenerative neuronal phenotype and sterility. On the basis of these results, we propose that huntingtin is required for neuronal function and survival in the brain and that a loss-of-function mechanism may contribute to HD pathogenesis.

Original languageEnglish (US)
Pages (from-to)300-306
Number of pages7
JournalNature Genetics
Volume26
Issue number3
DOIs
StatePublished - Nov 20 2000

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Huntington Disease
Infertility
Testis
Brain
Prosencephalon
Genetic Recombination
Phenotype
Mouse Hdh protein

All Science Journal Classification (ASJC) codes

  • Genetics

Cite this

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. / Dragatsis, Ioannis; Levine, Michael S.; Zeitlin, Scott.

In: Nature Genetics, Vol. 26, No. 3, 20.11.2000, p. 300-306.

Research output: Contribution to journalArticle

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