Neuroscience 2005 Abstract
| Presentation Number: | 89.7 |
|---|---|
| Abstract Title: | A role for p53 in Huntington's disease. |
| Authors: |
Bae, B.*1
; Xu, H.2
; Montell, C.1,2
; Ross, C. A.1,3,4
; Snyder, S. H.1,3,5
; Sawa, A.1,3
1Neurosci., Johns Hopkins Univ. School of Medicine, Baltimore, MD 2Biological Chemistry, Johns Hopkins Univ. School of Medicine, Baltimore, MD 3Psychiatry and Behavioral Sciences, Johns Hopkins Univ. School of Medicine, Baltimore, MD 4Neurology, Johns Hopkins Univ. School of Medicine, Baltimore, MD 5MD, 725 N. Wolfe Street, WBSB 813, 21205, |
| Primary Theme and Topics |
Disorders of the Nervous System - Neurodegenerative and Movement Disorders -- Trinucleotide repeat diseases: Huntington's disease--disease mechanisms |
| Session: |
89. Huntington's Disease: Mechanisms I Poster |
| Presentation Time: | Saturday, November 12, 2005 3:00 PM-4:00 PM |
| Location: | Washington Convention Center - Hall A-C, Board # PP12 |
| Keywords: | Huntington's disease, p53 |
Huntington's disease is a progressive neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) repeat in the huntingtin protein. We previously reported a role of p53 in the mitochondria-associated cellular dysfunction and behavioral abnormalities of Huntington’s disease (HD) (Bae et al, SFN abstract 2003). Here we present new evidences further supporting a crucial role of p53 in HD. First, we confirmed in vitro protein binding of Huntingtin (Htt) to p53 in a polyQ-dependent manner. Consistently, both endogenous N-terminal fragment and full length Htt co-precipitate with p53. Transcriptional activity of p53 is augmented by mutant Htt (mHtt) in the nucleus, resulting in upregulation of several p53 target proteins that are functionally associated with mitochondria, including Bax and Puma. In addition to the normalization of behavioral abnormalities in mHtt transgenic (mHtt-Tg) mice by genetic deletion of p53, mHtt-Tg flies in a p53 null background are resistant to mHtt-induced neurodegeneration in the retina. These results further support a crucial role for p53 in HD pathogenesis.
Supported by NIH, HDF
Sample Citation:
[Authors]. [Abstract Title]. Program No. XXX.XX. 2005 Neuroscience Meeting Planner. Washington, DC: Society for Neuroscience, 2005. Online.
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