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Bone Marrow Transplantation Confers Modest Benefits in Mouse Models of Huntington's Disease.

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Author

  • Wanda Kwan
  • Anna Magnusson-Lind
  • Austin Chou
  • Anthony Adame
  • Monica J Carson
  • Shinichi Kohsaka
  • Eliezer Masliah
  • Thomas Möller
  • Richard Ransohoff
  • Sarah J Tabrizi
  • Maria Björkqvist
  • Paul J Muchowski
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Summary, in English

Huntington's disease (HD) is caused by an expanded polyglutamine tract in the protein huntingtin (htt). Although HD has historically been viewed as a brain-specific disease, htt is expressed ubiquitously, and recent studies indicate that mutant htt might cause changes to the immune system that could contribute to pathogenesis. Monocytes from HD patients and mouse models are hyperactive in response to stimulation, and increased levels of inflammatory cytokines and chemokines are found in pre-manifest patients that correlate with pathogenesis. In this study, wild-type (WT) bone marrow cells were transplanted into two lethally irradiated transgenic mouse models of HD that ubiquitously express full-length htt (YAC128 and BACHD mice). Bone marrow transplantation partially attenuated hypokinetic and motor deficits in HD mice. Increased levels of synapses in the cortex were found in HD mice that received bone marrow transplants. Importantly, serum levels of interleukin-6, interleukin-10, CXC chemokine ligand 1, and interferon-γ were significantly higher in HD than WT mice but were normalized in mice that received a bone marrow transplant. These results suggest that immune cell dysfunction might be an important modifier of pathogenesis in HD.

Publishing year

2012

Language

English

Pages

133-142

Publication/Series

The Journal of neuroscience : the official journal of the Society for Neuroscience

Volume

32

Issue

1

Document type

Journal article

Publisher

Society for Neuroscience

Topic

  • Neurosciences

Status

Published

Research group

  • Biomarkers in Brain Disease

ISBN/ISSN/Other

  • ISSN: 1529-2401