The lack of correlation between changes in mice lacking PGC-1α and R6/2 HD mice are not entirely surprising given that we were unable to replicate a previous report of decreased expression of PGC-1α in R6/2 striatum [23], a result further validated by a lack of change in transcript expression of the PGC-1α-dependent gene Pvalb. The gene discussed is PPARGC1A; the disease is ring chromosome 6.