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J. Biol. Chem., Vol. 276, Issue 18, 14545-14548, May 4, 2001
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,,
From the Department of Pharmacological Sciences, University of
Milano, Via Balzaretti 9, 20133 Milano, Italy
Huntington's Disease is an inherited
neurodegenerative disease that affects the medium spiny neurons in the
striatum. The disease is caused by the expansion of a polyglutamine
sequence in the N terminus of Huntingtin (Htt), a widely expressed
protein. Recently, we have found that Htt is an antiapoptotic protein
in striatal cells and acts by preventing caspase-3 activity. Here we
report that Htt overexpression in other CNS-derived cells can protect
them from more than 20 days exposure to fatal stimuli. In particular,
we found that cytochrome c continues to be released from
mitochondria into the cytosol of cells that overexpress normal Htt.
However, procaspase-9 is not processed, indicating that wild-type Htt
(wtHtt) acts downstream of cytochrome c release.
These data show that Htt inhibits neuronal cell death by
interfering with the activity of the apoptosome complex.
These authors contributed equally to this work.
§
A member of the Coalition for the Cure (H. D. S. A.) and of the
Cure Initiative (H. D. F.).
¶
To whom correspondence should be addressed. Tel.:
39-02-20488349; Fax: 39-02-29404961; E-mail:
elena.cattaneo@unimi.it.
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