p53 Mediates Cellular Dysfunction and Behavioral Abnormalities in Huntington’s Disease

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p53 Mediates Cellular Dysfunction and Behavioral Abnormalities in Huntington’s Disease

We present evidence for a specific role of p53 in the mitochondria-associated cellular dysfunction and behavioral abnormalities of Huntington's disease (HD). Mutant huntingtin (mHtt) with expanded polyglutamine (polyQ) binds to p53 and upregulates levels of nuclear p53 as well as p53 transcriptional activity in neuronal cultures. The augmentation is specific, as it occurs with mHtt but not muta...

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ژورنال

عنوان ژورنال: Neuron

سال: 2005

ISSN: 0896-6273

DOI: 10.1016/j.neuron.2005.06.005