Article
Mutant huntingtin: nuclear translocation and cytotoxicity mediated by GAPDH.
Proceedings of the National Academy of Sciences of the United States of America - 28 Feb 2006
Bae Byoung-Il, Hara Makoto R, Cascio Matthew B, Wellington Cheryl L, Hayden Michael R, Ross Christopher A, Ha Hyo Chol, Li Xiao-Jiang, Snyder Solomon H, Sawa Akira
Abstract excerpt
The pathophysiology of Huntington's disease reflects actions of mutant Huntingtin (Htt) (mHtt) protein with polyglutamine repeats, whose N-terminal fragment translocates to the nucleus to elicit neurotoxicity. We establish that the nuclear translocation and associated cytotoxicity of mHtt reflect a ternary complex of mHtt with GAPDH and Siah1, a ubiquitin-E3-ligase. Overexpression of GAPDH or Siah1 enhances...
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