Clinical and Developmental Immunology
Volume 2013 (2013), Article ID 541259, 11 pages
http://dx.doi.org/10.1155/2013/541259
Review Article
The Role of the Immune System in Huntington’s Disease
1Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, 44791 Bochum, Germany
2International Graduate School of Neuroscience, Ruhr-University Bochum, 44791 Bochum, Germany
3Department of Neurology, Friedrich-Alexander-University Erlangen, 91054 Erlangen, Germany
2International Graduate School of Neuroscience, Ruhr-University Bochum, 44791 Bochum, Germany
3Department of Neurology, Friedrich-Alexander-University Erlangen, 91054 Erlangen, Germany
Received 3 April 2013; Accepted 19 June 2013
Academic Editor: Dan Frenkel
Copyright © 2013 Gisa Ellrichmann et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Huntington’s disease (HD) is characterized by a progressive course of disease until death 15–20 years after the first symptoms occur and is caused by a mutation with expanded CAG repeats in the huntingtin (htt) protein. Mutant htt (mhtt) in the striatum is assumed to be the main reason for neurodegeneration. Knowledge about pathophysiology has rapidly improved discussing influences of excitotoxicity, mitochondrial damage, free radicals, and inflammatory mechanisms. Both innate and adaptive immune systems may play an important role in HD. Activation of microglia with expression of proinflammatory cytokines, impaired migration of macrophages, and deposition of complement factors in the striatum indicate an activation of the innate immune system. As part of the adaptive immune system, dendritic cells (DCs) prime T-cell responses secreting inflammatory mediators. In HD, DCs may contain mhtt which brings the adaptive immune system into the focus of interest. These data underline an increasing interest in the peripheral immune system for pathomechanisms of HD. It is still unclear if neuroinflammation is a reactive process or if there is an active influence on disease progression. Further understanding the influence of inflammation in HD using mouse models may open various avenues for promising therapeutic approaches aiming at slowing disease progression or forestalling onset of disease.
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