PPARGC1A and inborn mitochondrial metabolism disorder: Considering the spectrum of roles of PGC-1α in regulating and promoting mitochondrial functions and the fact that a number of genes involved in disease-causing mutations and/or that involved in modeling mitochondrial disease have direct or indirect interactions with PGC-1α (e.g., ANT-1, POLG1, Tfam, NRF-1, NRF-2, PPARs, ERRs, Mn-SOD, and CREB), the rationale for PGC-1α induction to provide symptomatic benefit in these currently intractable groups of diseases can be accepted [203].