PT  - JOURNAL ARTICLE
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
TI  - Adult Conditional Knockout of PGC-1α Leads to Loss of Dopamine Neurons
AID  - 10.1523/ENEURO.0183-16.2016
DP  - 2016 Jul 01
TA  - eneuro
PG  - ENEURO.0183-16.2016
VI  - 3
IP  - 4
4099  - http://www.eneuro.org/content/3/4/ENEURO.0183-16.2016.short
4100  - http://www.eneuro.org/content/3/4/ENEURO.0183-16.2016.full
SO  - eneuro2016 Jul 01; 3
AB  - 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.