Mutant huntingtin interferes with the PGC-1α transcriptional pathway, impairing its ability to activate downstream genes, while PGC-1α ectopic expression provides neuroprotection in transgenic HD mice and in the 3-NPA mouse model (Cui et al., 2006; Weydt et al., 2006). The gene discussed is PPARGC1A; the disease is Huntington disease.