PT  - JOURNAL ARTICLE
AU  - Dorotea Rigamonti
AU  - Johannes H. Bauer
AU  - Claudio De-Fraja
AU  - Luciano Conti
AU  - Simonetta Sipione
AU  - Clara Sciorati
AU  - Emilio Clementi
AU  - Abigail Hackam
AU  - Michael R. Hayden
AU  - Yong Li
AU  - Jillian K. Cooper
AU  - Christopher A. Ross
AU  - Stefano Govoni
AU  - Claudius Vincenz
AU  - Elena Cattaneo
TI  - Wild-Type Huntingtin Protects from Apoptosis Upstream of Caspase-3
AID  - 10.1523/JNEUROSCI.20-10-03705.2000
DP  - 2000 May 15
TA  - The Journal of Neuroscience
PG  - 3705--3713
VI  - 20
IP  - 10
4099  - http://www.jneurosci.org/content/20/10/3705.short
4100  - http://www.jneurosci.org/content/20/10/3705.full
SO  - J. Neurosci.2000 May 15; 20
AB  - 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.