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Astrocytes in Huntington’s Disease

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Neuroglia in Neurodegenerative Diseases

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1175))

Abstract

Huntington’s disease (HD) is a dominantly inherited neurodegenerative disease that results in motor, cognitive and psychiatric dysfunction. It is caused by a polyglutamine repeat expansion mutation in the widely expressed HTT protein. The clinical manifestations of HD have been largely attributed to the neurodegeneration of specific neuronal cell types in the brain. However, it has become clear that other cell types, including astrocytes, play important roles in the pathogenesis of HD. The mutant HTT (mHTT) protein is present in neuronal and non-neuronal cell types throughout the nervous system. Studies designed to understand the contribution of mHTT expression in non-neuronal cell types to HD pathogenesis has lagged considerably behind those focused on neurons. However, the role of astrocytes in HD has received more attention over the last 5–10 years. In this chapter we present an overview of HD and our current understanding of astrocytic involvement in this disease. We describe the neuropathological features of HD and provide evidence of morphological and molecular changes in mHTT expressing astrocytes. We review data from animal models and HD patients that implicate mHTT expressing astrocytes to the progression of HD.

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Acknowledgements

The authors’ work is supported by grants from the Dixon Family Foundation, the Hereditary Disease Foundation and the National Institutes of Health.

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  1. Department of Neurology and Center for Neurodegeneration and Experimental Therapeutics, University of Alabama at Birmingham, 1720 2nd Ave S, CIRC 425B, Birmingham, AL 35294, USA

    Michelle Gray

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  1. Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK

    Alexei Verkhratsky

  2. School of Life Science and Technology, ShanghaiTech University, Shanghai, China

    Margaret S. Ho

  3. Institute of Pathophysiology, University of Ljubljana, Ljubljana, Slovenia

    Robert Zorec

  4. Department of Neurobiology, The University of Alabama at Birmingham, Birmingham, AL, USA

    Vladimir Parpura

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Gray, M. (2019). Astrocytes in Huntington’s Disease. In: Verkhratsky, A., Ho, M., Zorec, R., Parpura, V. (eds) Neuroglia in Neurodegenerative Diseases. Advances in Experimental Medicine and Biology, vol 1175. Springer, Singapore. https://doi.org/10.1007/978-981-13-9913-8_14

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