Ontology highlight
ABSTRACT:
SUBMITTER: Holter SM
PROVIDER: S-EPMC3838378 | biostudies-literature | 2013
REPOSITORIES: biostudies-literature
Hölter Sabine M SM Stromberg Mary M Kovalenko Marina M Garrett Lillian L Glasl Lisa L Lopez Edith E Guide Jolene J Götz Alexander A Hans Wolfgang W Becker Lore L Rathkolb Birgit B Rozman Jan J Schrewed Anja A Klingenspor Martin M Klopstock Thomas T Schulz Holger H Wolf Eckhard E Wursta Wolfgang W Gillis Tammy T Wakimoto Hiroko H Seidman Jonathan J MacDonald Marcy E ME Cotman Susan S Gailus-Durner Valérie V Fuchs Helmut H de Angelis Martin Hrabě MH Lee Jong-Min JM Wheeler Vanessa C VC
PloS one 20131122 11
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by the expansion of a CAG trinucleotide repeat in the HTT gene encoding huntingtin. The disease has an insidious course, typically progressing over 10-15 years until death. Currently there is no effective disease-modifying therapy. To better understand the HD pathogenic process we have developed genetic HTT CAG knock-in mouse models that accurately recapitulate the HD mutation in man. Here, we describe results o ...[more]