Article
C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.
Neuron - 4 May 2016
Liu Yuanjing, Pattamatta Amrutha, Zu Tao, Reid Tammy, Bardhi Olgert, Borchelt David R, Yachnis Anthony T, Ranum Laura P W
Abstract excerpt
To define how the C9orf72 GGGGCC expansion mutation causes ALS/FTD and to facilitate therapy development, a mouse model that recapitulates the molecular and phenotypic features of the disease is urgently needed. Two groups recently reported BAC mouse models that produce RNA foci and RAN proteins but, surprisingly, do not develop the neurodegenerative or behavioral features of ALS/FTD. We now report a BAC mouse...
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