ALS-causing mutations differentially affect PGC-1alpha expression and function in the brain vs. peripheral tissues

Amyotrophic later sclerosis is a motor neuron disease accompanied by metabolic changes. PGC (PPAR gamma coactivator)-1alpha is a master regulator of mitochondrial biogenesis and function and of critical importance for all metabolically active tissues. PGC-1alpha is a genetic modifier of ALS. We used microarray analysis to identify PGC-1alpha target genes in the brain. Total mRNA from striata of three homozygous B6.129-Ppargc1atm1Brsp/J and three wild type littermates were used for the analysis.