Skip to main content
. Author manuscript; available in PMC: 2014 Aug 4.
Published in final edited form as: Annu Rev Genomics Hum Genet. 2011;12:301–325. doi: 10.1146/annurev-genom-082410-101440

Figure 4. Parkin controls the expression of PGC-1α through PARIS.

Figure 4

PARIS is a key pathogenic substrate of Parkin that accumulates in models of Parkin inactivation and in patients with Parkin mutations and PD patients due to nitrosative (NO), reactive oxygen species (ROS) and dopamine (DA) stress as well as c-Abl phosphorylation. PARIS is a transcriptional repressor that selectively down regulates PGC1-α by binding to insulin response sequences (IRS) in the PGC1-α promoter leading to downregulation of NRF-1, which transcriptionally controls mitochondrial biogenesis leading to DA neuron degeneration.