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Trends Cell Biol. 2015 Mar;25(3):158-70. doi: 10.1016/j.tcb.2014.11.002. Epub 2014 Dec 11.

Protecting the mitochondrial powerhouse.

Author information

1
Laboratory of Molecular Gerontology, National Institute on Aging (NIA), National Institutes of Health (NIH), Baltimore, MD 21224, USA.
2
Laboratory of Molecular Gerontology, National Institute on Aging (NIA), National Institutes of Health (NIH), Baltimore, MD 21224, USA. Electronic address: vbohr@nih.gov.

Abstract

Mitochondria are the oxygen-consuming power plants of cells. They provide a critical milieu for the synthesis of many essential molecules and allow for highly efficient energy production through oxidative phosphorylation. The use of oxygen is, however, a double-edged sword that on the one hand supplies ATP for cellular survival, and on the other leads to the formation of damaging reactive oxygen species (ROS). Different quality control pathways maintain mitochondria function including mitochondrial DNA (mtDNA) replication and repair, fusion-fission dynamics, free radical scavenging, and mitophagy. Further, failure of these pathways may lead to human disease. We review these pathways and propose a strategy towards a treatment for these often untreatable disorders.

KEYWORDS:

DNA repair; disease; mitochondria; mitophagy; reactive oxygen species

PMID:
25499735
PMCID:
PMC5576887
DOI:
10.1016/j.tcb.2014.11.002
[Indexed for MEDLINE]
Free PMC Article

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