In addition to being required for the expression of the genes involved in oxidative phosphorylation, PGC-1α is necessary for the expression of the PV-IN-enriched transcripts for neurotransmitter release and axonal integrity, including Nefh [110,111,112,292], whose protein product is used as a biomarker of axonal damage in ALS [293,294]. The gene discussed is PPARGC1A; the disease is amyotrophic lateral sclerosis.