• Significant increase in plasma levels of IL-6, IL-8, IL-4, IL-10, and TNF-α in HD mouse models and patients • Increased IL-6, IL-8 and TNF-α in the HD CSF and postmortem HD striatal tissue • Remarkable correlation between plasma TNF-α levels and UHDRS chorea scores, motor scores, and total functional capacity • Monocytes, macrophages, and microglia from HD mouse models and patients express mHtt and dis-played functional over-activity when stimulated with LPS and IFN-γ. Here, IL10 is linked to Huntington disease.