Article
In vivo cell-autonomous transcriptional abnormalities revealed in mice expressing mutant huntingtin in striatal but not cortical neurons.
Human molecular genetics - 15 Mar 2011
Thomas Elizabeth A, Coppola Giovanni, Tang Bin, Kuhn Alexandre, Kim SoongHo, Geschwind Daniel H, Brown Timothy B, Luthi-Carter Ruth, Ehrlich Michelle E
Abstract excerpt
Huntington's disease (HD), caused by a CAG repeat expansion in the huntingtin (HTT) gene, is characterized by abnormal protein aggregates and motor and cognitive dysfunction. Htt protein is ubiquitously expressed, but the striatal medium spiny neuron (MSN) is most susceptible to dysfunction and death. Abnormal gene expression represents a core pathogenic feature of HD, but the relative roles of cell-autonomous...
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