NFKB1 and Huntington disease: In support of this hypothesis, a few studies showed that decreasing glia activation and production of pro-inflammatory cytokines has beneficial effects in HD mouse models: the knock-out of TLR2 or TLR4 extended the life-span of N171-82Q mice [45], a model that overexpresses an N-terminal fragment of mutant HTT in neurons only [109], while inhibition of IKK and the NFkB pathway [110] or TNF signaling [79] in R6/2 mice decreased neurodegeneration and improved mouse behaviour.