Its potential relevance in HD has been suggested by a specific decrease in PGC-1α in the postmortem caudate nucleus of HD patients, a similar motor phenotype in PGC-1α knockout and HD mice that is worsened when combining both mutations and by the fact that overexpression experiments restoring PGC-1α levels ameliorated neurodegeneration and mHtt aggregation [104, 120]. This evidence concerns the gene PPARGC1A and Huntington disease.