@article {JiangENEURO.0183-16.2016, author = {Haisong Jiang and Sung-Ung Kang and Shuran Zhang and Senthilkumar Karuppagounder and Jinchong Xu and Yong-Kyu Lee and Bong-Gu Kang and Yunjong Lee and Jianmin Zhang and Olga Pletnikova and Juan C. Troncoso and Shelia Pirooznia and Shaida A. Andrabi and Valina L. Dawson and Ted M. Dawson}, title = {Adult Conditional Knockout of PGC-1α Leads to Loss of Dopamine Neurons}, volume = {3}, number = {4}, elocation-id = {ENEURO.0183-16.2016}, year = {2016}, doi = {10.1523/ENEURO.0183-16.2016}, publisher = {Society for Neuroscience}, abstract = {Parkinson{\textquoteright}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.}, URL = {https://www.eneuro.org/content/3/4/ENEURO.0183-16.2016}, eprint = {https://www.eneuro.org/content/3/4/ENEURO.0183-16.2016.full.pdf}, journal = {eNeuro} }