Article
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity.
Nature medicine - 1 Mar 2011
Song Wenjun, Chen Jin, Petrilli Alejandra, Liot Geraldine, Klinglmayr Eva, Zhou Yue, Poquiz Patrick, Tjong Jonathan, Pouladi Mahmoud A, Hayden Michael R, Masliah Eliezer, Ellisman Mark, Rouiller Isabelle, Schwarzenbacher Robert, Bossy Blaise, Perkins Guy, Bossy-Wetzel Ella
Abstract excerpt
Huntington's disease is an inherited and incurable neurodegenerative disorder caused by an abnormal polyglutamine (polyQ) expansion in huntingtin (encoded by HTT). PolyQ length determines disease onset and severity, with a longer expansion causing earlier onset. The mechanisms of mutant huntingtin-mediated neurotoxicity remain unclear; however, mitochondrial dysfunction is a key event in Huntington's disease...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
