The huntingtin (HTT) gene, which is mutated in Huntington's disease, is expressed in non-neuronal cells as well as neurons. Here, both microglia and peripheral immune cells from newborn mutant Htt mice showed markedly impaired migration, and this impairment could be reproduced by expressing a mutant Htt fragment in microglial cell lines. Peripheral immune cells from pre-symptomatic individuals with the Huntington's disease mutation also showed impaired migration, suggesting that these immune changes contribute to the development of the disease.
ORIGINAL RESEARCH PAPER
Kwan, W. et al. Mutant huntingtin impairs immune cell migration in Huntington disease. J. Clin. Invest. 19 Nov 2012 (doi:10.1172/JCI64484)
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Jones, R. Immune cell migration in Huntington's disease. Nat Rev Neurosci 14, 2 (2013). https://doi.org/10.1038/nrn3412
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DOI: https://doi.org/10.1038/nrn3412