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Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction.

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Author

Summary, in English

Huntington's disease (HD) is a hereditary neurodegenerative disorder characterized by progressive psychiatric, cognitive, and motor disturbances. We studied the expression of synaptic vesicle proteins in the R6/1 transgenic mouse model of HD. We observed that the levels of rabphilin 3A, a protein involved in exocytosis, is substantially decreased in synapses of most brain regions in R6/1 mice. The appearance of the reduction coincides with the onset of motor deficits and behavioral disturbances. Double immunohistochemistry did not show colocalization between rabphilin 3A and huntingtin aggregates in the HD mice. Using in situ hybridization, we demonstrated that rabphilin 3A mRNA expression was substantially reduced in the R6/1 mouse cortex compared to wild-type mice. Our results indicate that a decrease in mRNA levels underlie the depletion of protein levels of rabphilin 3A, and we suggest that this reduction may be involved in causing impaired synaptic transmission in R6/1 mice.

Publishing year

2005

Language

English

Pages

673-684

Publication/Series

Neurobiology of Disease

Volume

20

Issue

3

Document type

Journal article

Publisher

Elsevier

Topic

  • Neurosciences

Keywords

  • vesicle protein
  • huntingtin
  • synaptic dysfunction
  • synapse
  • mouse
  • Huntington's disease
  • R6/1
  • CAG repeal
  • neurodegeneration
  • polyglutamine
  • rabphilin
  • exocytosis

Status

Published

ISBN/ISSN/Other

  • ISSN: 0969-9961