Ontology highlight
ABSTRACT:
ORGANISM(S): Mus musculus
DISEASE(S): normal
SUBMITTER: Marcy MacDonald
PROVIDER: E-GEOD-3583 | biostudies-arrayexpress |
REPOSITORIES: biostudies-arrayexpress
Lee Jong-Min JM Ivanova Elena V EV Seong Ihn Sik IS Cashorali Tanya T Kohane Isaac I Gusella James F JF MacDonald Marcy E ME
PLoS genetics 20070627 8
The Huntington's disease (HD) CAG repeat, encoding a polymorphic glutamine tract in huntingtin, is inversely correlated with cellular energy level, with alleles over approximately 37 repeats leading to the loss of striatal neurons. This early HD neuronal specificity can be modeled by respiratory chain inhibitor 3-nitropropionic acid (3-NP) and, like 3-NP, mutant huntingtin has been proposed to directly influence the mitochondrion, via interaction or decreased PGC-1alpha expression. We have teste ...[more]