Reduced full-length PGC-1α expression has been repeatedly found in postmortem brain samples from HD patients as well as in transgenic mouse models [241], while levels of the N-truncated splice variant of PGC1α (NT-PGC1α) were found to be upregulated in the human HD brain, and striatal cells of mouse HD models [242]. This evidence concerns the gene PPARGC1A and Huntington disease.