Skip NavigationSkip to Content
Return Return to Search Results

Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA

  1. Author:
    Schilling, G.
    Jinnah, H. A.
    Gonzales, V.
    Coonfield, M. L.
    Kim, Y.
    Wood, J. D.
    Price, D. L.
    Li, X. J.
    Jenkins, N.
    Copeland, N.
    Moran, T.
    Ross, C. A.
    Borchelt, D. R.

  2. Author Address

    Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA. Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA. Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA. Johns Hopkins Univ, Sch Med, Dept Psychiat & Behav Sci, Baltimore, MD 21205 USA. Johns Hopkins Univ, Sch Med, Dept Neurosci, Baltimore, MD 21205 USA. Emory Univ, Sch Med, Dept Genet, Atlanta, GA 30322 USA. NCI, Frederick Canc Res & Dev Ctr, Mouse Canc Genet Program, Frederick, MD 21702 USA. Schilling G Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA.
    1. Year: 2001
  2. Journal: Neurobiology of Disease
    1. 8
    2. 3
    3. Pages: 405-418
  4. Type of Article: Article
  1. Abstract:

    Huntington's disease (HD) and Dentatorubral and pallidoluysian atrophy (DRPLA) are autosomal dominant, neurodegenerative disorders caused by the expansion of polyglutamine tracts in their respective proteins, huntingtin and atrophin-1. We have previously generated mouse models of these disorders, using transgenes expressed via the prion protein promoter. Here, we report the first direct comparison of abnormalities in these models. The Ho mice show abbreviated lifespans (4-6 months), hypoactivity, and mild impairment of motor skills. The DRPLA mice show severe tremors, are hyperactive, and are profoundly uncoordinated. Neuropathological analyses reveal that the distribution of diffuse nuclear immunolabeling and neuronal intranuclear inclusions (NII's), in the CNS of both models, was remarkably similar. Cytoplasmic aggregates of huntingtin were the major distinguishing neuropathological feature of the Ho mice; mutant atrophin-1 accumulated/aggregated only in the nucleus. We suggest that the distinct behavioral and neuropathological phenotypes in these mice reflect differences in the way these mutant proteins perturb neuronal function. (C) 2001 Academic Press.

    See More

  1. Keywords:

External Sources

  1. No sources found.

Library Notes

  1. No notes added.
NCI at Frederick

You are leaving a government website.

This external link provides additional information that is consistent with the intended purpose of this site. The government cannot attest to the accuracy of a non-federal site.

Linking to a non-federal site does not constitute an endorsement by this institution or any of its employees of the sponsors or the information and products presented on the site. You will be subject to the destination site's privacy policy when you follow the link.

ContinueCancel