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PGC-1α activation as a therapeutic approach in mitochondrial disease

✍ Scribed by Tina Wenz

Publisher
John Wiley and Sons
Year
2009
Tongue
English
Weight
381 KB
Volume
61
Category
Article
ISSN
1521-6543

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✦ Synopsis

Abstract

Mitochondria play a central role in cellular homeostasis. Hence, mitochondrial dysfunction resulting in impaired ATP supply is detrimental to cell viability and causes severe and often fatal disorders. Current treatment of these mitochondrial disorders is limited and mostly addresses the symptoms, but not the deficiency itself. Recent work in cell and animal models of mitochondrial disease shows that increased mitochondrial biogenesis can boost residual OXPHOS capacity and thereby prevent the bioenergetic crisis. In animal models, this strategy results in increased health and lifespan. Here, I review which mitochondrial processes are affected in known mitochondrial disorders and discuss why PGC‐1α mediated mitochondrial biogenesis offers a promising venue for treatment of mitochondrial disorders. © 2009 IUBMB IUBMB Life, 61(11): 1051–1062, 2009

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