Article
Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease.
Proceedings of the National Academy of Sciences of the United States of America - 14 Oct 2003
Trushina Eugenia, Heldebrant Michael P, Perez-Terzic Carmen M, Bortolon Ryan, Kovtun Irina V, Badger John D, Terzic Andre, Estévez Alvaro, Windebank Anthony J, Dyer Roy B, Yao Janet, McMurray Cynthia T
Abstract excerpt
There has been a longstanding debate regarding the role of proteolysis in Huntington's disease. The toxic peptide theory posits that N-terminal cleavage fragments of mutant Huntington's disease protein [mutant huntingtin (mhtt)] enter the nucleus to cause transcriptional dysfunction. However, recent data suggest a second model in which proteolysis of full-length mhtt is inhibited. Importantly, the two competing...
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