Innate immune activation and aberrant function in the R6/2 mouse model and Huntington's disease iPSC-derived microglia.

ABSTRACT: Huntington's disease (HD) is an inherited autosomal dominant neurodegenerative disease caused by CAG repeats in exon 1 of the HTT gene. A hallmark of HD along with other psychiatric and neurodegenerative diseases is alteration in the neuronal circuitry and synaptic loss. Microglia and peripheral innate immune activation have been reported in pre-symptomatic HD patients; however, what "activation" signifies for microglial and immune function in HD and how it impacts synaptic health remains unclear. In this study we sought to fill these gaps by capturing immune phenotypes and functional activation states of microglia and peripheral immunity in the R6/2 model of HD at pre-symptomatic, symptomatic and end stages of disease. These included characterizations of microglial phenotypes at si

SUBMITTER: Gasser J 

PROVIDER: S-EPMC10319581 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature