PPARGC1A and Huntington disease: Combined with reduced expression of PGC-1α target genes involved in energy production in BAT, reduced PGC-1α activity possibly leads to a global defect in mitochondrial function in HD.470 At the tissue level, PGC-1α plays a role in postnatal myelination by regulating the expression of myelin basic protein (MBP) and cholesterol synthesis in HD.