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To address the role of these cells in disease pathogenesis, we depleted microglia from R6\/2 mice, a rapidly progressing model of Huntington\u2019s disease marked by behavioural impairment, mutant huntingtin (mHTT) accumulation, and early death, through colony-stimulating factor 1 receptor inhibition (CSF1Ri) with pexidartinib (PLX3397) for the duration of disease. Although we observed an interferon gene signature in addition to downregulated neuritogenic and synaptic gene pathways with disease, overt inflammation was not evident by microglial morphology or cytokine transcript levels in R6\/2 mice. Nonetheless, CSF1Ri-induced microglial elimination reduced or prevented disease-related grip strength and object recognition deficits, mHTT accumulation, astrogliosis, and striatal volume loss, the latter of which was not associated with reductions in cell number but with the extracellular accumulation of chondroitin sulphate proteoglycans (CSPGs)\u2014a primary component of glial scars. A concurrent loss of proteoglycan-containing perineuronal nets was also evident in R6\/2 mice, and microglial elimination not only prevented this but also strikingly increased perineuronal nets in the brains of na\u00efve littermates, suggesting a new role for microglia as homeostatic regulators of perineuronal net formation and integrity.<\/jats:p>","DOI":"10.1093\/brain\/awz363","type":"journal-article","created":{"date-parts":[[2019,11,5]],"date-time":"2019-11-05T15:15:00Z","timestamp":1572966900000},"page":"266-288","source":"Crossref","is-referenced-by-count":161,"title":["Microglial depletion prevents extracellular matrix changes and striatal volume reduction in a model of Huntington's disease"],"prefix":"10.1093","volume":"143","author":[{"given":"Joshua D","family":"Crapser","sequence":"first","affiliation":[{"name":"Department of Neurobiology and Behavior, University of California, Irvine (UCI), Irvine, CA, USA"},{"name":"Institute of Memory Impairments and Neurological 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USA"},{"name":"Institute of Memory Impairments and Neurological Disorders, University of California, Irvine, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie M","family":"Thompson","sequence":"additional","affiliation":[{"name":"Department of Neurobiology and Behavior, University of California, Irvine (UCI), Irvine, CA, USA"},{"name":"Institute of Memory Impairments and Neurological Disorders, University of California, Irvine, CA, USA"},{"name":"Department of Psychiatry and Human Behavior, University of California, Irvine, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim N","family":"Green","sequence":"additional","affiliation":[{"name":"Department of Neurobiology and Behavior, University of California, Irvine (UCI), Irvine, CA, USA"},{"name":"Institute of Memory Impairments and Neurological Disorders, University of California, Irvine, CA, 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