TY - JOUR T1 - Adult Conditional Knockout of PGC-1α Leads to Loss of Dopamine Neurons JF - eneuro JO - eneuro DO - 10.1523/ENEURO.0183-16.2016 VL - 3 IS - 4 SP - ENEURO.0183-16.2016 AU - Haisong Jiang AU - Sung-Ung Kang AU - Shuran Zhang AU - Senthilkumar Karuppagounder AU - Jinchong Xu AU - Yong-Kyu Lee AU - Bong-Gu Kang AU - Yunjong Lee AU - Jianmin Zhang AU - Olga Pletnikova AU - Juan C. Troncoso AU - Shelia Pirooznia AU - Shaida A. Andrabi AU - Valina L. Dawson AU - Ted M. Dawson Y1 - 2016/07/01 UR - http://www.eneuro.org/content/3/4/ENEURO.0183-16.2016.abstract N2 - Parkinson’s disease (PD) is a chronic progressive neurodegenerative disorder. Recent studies have implicated a role for peroxisome proliferator-activated receptor γ coactivator protein-1α (PGC-1α) in PD and in animal or cellular models of PD. The role of PGC-1α in the function and survival of substantia nigra pars compacta (SNpc) dopamine neurons is not clear. Here we find that there are four different PGC-1α isoforms expressed in SH-SY5Y cells, and these four isoforms are expressed across subregions of mouse brain. Adult conditional PGC-1α knock-out mice show a significant loss of dopaminergic neurons that is accompanied by a reduction of dopamine in the striatum. In human PD postmortem tissue from the SNpc, there is a reduction of PGC-1α isoforms and mitochondria markers. Our findings suggest that all four isoforms of PGC-1α are required for the proper expression of mitochondrial proteins in SNpc DA neurons and that PGC-1α is essential for SNpc DA neuronal survival, possibly through the maintenance of mitochondrial function. ER -