Article
Progressive axonal transport and synaptic protein changes correlate with behavioral and neuropathological abnormalities in the heterozygous Q175 KI mouse model of Huntington's disease.
Human molecular genetics - 1 Sept 2014
Smith Gaynor A, Rocha Emily M, McLean Jesse R, Hayes Melissa A, Izen Sarah C, Isacson Ole, Hallett Penelope J
Abstract excerpt
A long-term goal of modeling Huntington's disease (HD) is to recapitulate the cardinal features of the disease in mice that express both mutant and wild-type (WT) huntingtin (Htt), as HD commonly manifests as a heterozygous condition in humans, and loss of WT Htt is associated with loss-of-function. In a new heterozygous Q175 knock-in (KI) mouse model, we performed an extensive evaluation of motor and cognitive...
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