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See 1 citation in The Journal Of Neuroscience 2000 by Rigamonti:

J Neurosci. 2000 May 15;20(10):3705-13.

Wild-type huntingtin protects from apoptosis upstream of caspase-3.

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Institute of Pharmacological Sciences, University of Milano, 20133 Milano, Italy.


Expansion of a polyglutamine sequence in the N terminus of huntingtin is the gain-of-function event that causes Huntington's disease. This mutation affects primarily the medium-size spiny neurons of the striatum. Huntingtin is expressed in many neuronal and non-neuronal cell types, implying a more general function for the wild-type protein. Here we report that wild-type huntingtin acts by protecting CNS cells from a variety of apoptotic stimuli, including serum withdrawal, death receptors, and pro-apoptotic Bcl-2 homologs. This protection may take place at the level of caspase-9 activation. The full-length protein also modulates the toxicity of the poly-Q expansion. Cells expressing full-length mutant protein are susceptible to fewer death stimuli than cells expressing truncated mutant huntingtin.

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