Abstract
Huntington’s disease (HD) is a debilitating inherited neurodegenerative disorder characterized by motor, cognitive and psychiatric deficits. Microglial and astrocyte activation, part of the process termed neuroinflammation, is one hallmark of HD, and modulation of neuroinflammation has been suggested as a potential target for therapeutic intervention. Although the relationship between neuroinflammation markers and the disease pathology is not completely understood, there is now compelling evidence to suggest that microglial and astrocyte activation signatures, identified as soluble factors in the cerebrospinal fluid or blood, or identified using PET imaging, could be used as potential complementary biomarkers to monitor and evaluate disease progression in HD patients. Identification of neuroinflammation markers prior to clinical symptoms opens up the possibility of evaluating disease-modifying treatments in the premanifest phase. Hence, neuroinflammatory biofluid and imaging biomarkers could provide an objective measurement for assessing HD severity and would also be valuable in the clinical care of existing HD patients. Neuroinflammatory biomarkers would also be useful in a clinical trial context, potentially serving as surrogate endpoints. This chapter will explore the evidence of roles for activated microglia, astrocytes, and peripheral immune cells in HD, and explore possible biomarkers of neuroinflammation.
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Lee, J.D., Lo, M.W., Fung, J.N.T., Woodruff, T.M. (2022). Neuroinflammation in Huntington’s Disease . In: Peplow, P.V., Martinez, B., Gennarelli, T.A. (eds) Neurodegenerative Diseases Biomarkers. Neuromethods, vol 173. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-1712-0_9
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