Ontology highlight
ABSTRACT:
SUBMITTER: Trushina E
PROVIDER: S-EPMC218731 | biostudies-literature | 2003 Oct
REPOSITORIES: biostudies-literature
Trushina Eugenia E Heldebrant Michael P MP Perez-Terzic Carmen M CM Bortolon Ryan R Kovtun Irina V IV Badger John D JD Terzic Andre A Estévez Alvaro A Windebank Anthony J AJ Dyer Roy B RB Yao Janet J McMurray Cynthia T CT
Proceedings of the National Academy of Sciences of the United States of America 20031003 21
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 theories differ with respect to subcellular distribution of mhtt at initiation of tox ...[more]