Article
Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation.
Human molecular genetics - 1 Mar 2017
Clayton Emma L, Mancuso Renzo, Nielsen Troels Tolstrup, Mizielinska Sarah, Holmes Holly, Powell Nicholas, Norona Frances, Larsen Jytte Overgaard, Milioto Carmelo, Wilson Katherine M, Lythgoe Mark F, Ourselin Sebastian, Nielsen Jörgen E, Johannsen Peter, Holm Ida, Collinge John, Oliver Peter L, Gomez-Nicola Diego, Isaacs Adrian M
Abstract excerpt
Frontotemporal dementia (FTD)-causing mutations in the CHMP2B gene lead to the generation of mutant C-terminally truncated CHMP2B. We report that transgenic mice expressing endogenous levels of mutant CHMP2B developed late-onset brain volume loss associated with frank neuronal loss and FTD-like changes in social behaviour. These data are the first to show neurodegeneration in mice expressing mutant CHMP2B and...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.