PGC-1α, a New Therapeutic Target in Huntington's Disease?

The coactivator PGC-1α is a key regulator of mitochondrial biogenesis and respiration, mediating expression of several transcription factors required for these programs. Three new studies reveal that PGC-1α expression is downregulated in patients with Huntington's disease (HD) and in several animal models of this neurodegenerative disorder, implicating PGC-1α in HD pathogenesis and providing a connection between impaired energy metabolism and neurodegeneration (Cui et al., 2006, Weydt et al., 2006 and St-Pierre et al., 2006).