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The role of immunity in Huntington's disease

Molecular Psychiatry volume 16, pages 889–902 (2011)Cite this article

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Abstract

Huntington's disease (HD) is a devastating and incurable neurodegenerative disorder characterized by progressive cognitive, psychiatric and motor impairments. Although the disease has been seen as a disorder purely of the brain, there is now emerging evidence that abnormalities outside the central nervous system are commonly seen in HD. Indeed, the mutant huntingtin (mHtt) coded for by the abnormal gene in HD is found in every cell type where its presence has been sought. In particular, there are a number of recent observations in HD patients that mHtt interacts with the immune system with accumulating evidence that changes in the immune system may critically contribute to the pathology of HD. However, the nature of this contribution remains unclear, to the extent that it is not even known whether the immune system has a beneficial or detrimental role in HD patients. In this review, we attempt to bring a novel understanding to the interaction of the immune system to HD pathology, thereby shedding light on its potential pathogenic role. As part of this discussion, we revisit the clinical data on the anti-inflammatory drug trials in HD and propose new experimental approaches to interrogate the role of immunity in this currently incurable disorder.

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Acknowledgements

We thank the reviewers for their insightful comments and Dr Richard Poulin from the CRCHUL for his valuable editing. Special thanks to Martine Saint-Pierre for her assistance in the preparation of this paper.

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  1. Axe Neurosciences, Centre de Recherche du CHUL (CHUQ), Boulevard Laurier, Québec, QC, Canada

    D Soulet & F Cicchetti

  2. Département de Psychiatrie et Neurosciences, Université Laval, Québec, QC, Canada

    D Soulet & F Cicchetti

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Soulet, D., Cicchetti, F. The role of immunity in Huntington's disease. Mol Psychiatry 16, 889–902 (2011). https://doi.org/10.1038/mp.2011.28

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