A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin

Huntington's disease (HD) is a neurodegenerative disorder caused by an abnormal expansion of a CAG repeat encoding a polyglutamine tract in the huntingtin (Htt) protein. The mutation leads to neuronal death through mechanisms which are still unknown. One hypothesis is that mitochondrial defects...

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Hauptverfasser: Damiano, Maria, Diguet, Elsa, Malgorn, Carole, D'Aurelio, Marilena, Galvan, Laurie, Petit, Fanny, Benhaim, Lucile, Guillermier, Martine, Houitte, Diane, Dufour, Noelle, Hantraye, Philippe, Canals, Josep M., Alberch, Jordi, Delzescaux, Thierry, Déglon, Nicole, Beal, M. Flint, Brouillet, Emmanuel
Format: Artigo
Sprache:Inglês
Veröffentlicht: Oxford University Press 2013
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Articles
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3766181/
https://ncbi.nlm.nih.gov/pubmed/23720495
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddt242
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