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DNA repair in organelles: Pathways, organization, regulation, relevance in disease and aging

Lookup NU author(s): Dr Pierre Boesch, Professor Robert Lightowlers


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Both endogenous processes and exogenous physical and chemical sources generate deoxyribonucleic acid (DNA) damage in the nucleus and organelles of living cells. To prevent deleterious effects, damage is balanced by repair pathways. DNA repair was first documented for the nuclear compartment but evidence was subsequently extended to the organelles. Mitochondria and chloroplasts possess their own repair processes. These share a number of factors with the nucleus but also rely on original mechanisms. Base excision repair remains the best characterized. Repair is organized with the other DNA metabolism pathways in the organelle membrane-associated nucleoids. DNA repair in mitochondria is a regulated, stress-responsive process. Organelle genomes do not encode DNA repair enzymes and translocation of nuclear-encoded repair proteins from the cytosol seems to be a major control mechanism. Finally, changes in the fidelity and efficiency of mitochondrial DNA repair are likely to be involved in DNA damage accumulation, disease and aging. The present review successively addresses these different issues. (C) 2010 Elsevier B.V. All rights reserved.

Publication metadata

Author(s): Boesch P, Weber-Lotfi F, Ibrahim N, Tarasenko V, Cosset A, Paulus F, Lightowlers RN, Dietrich A

Publication type: Review

Publication status: Published

Journal: Biochimica et Biophysica Acta: Molecular Cell Research

Year: 2011

Volume: 1813

Issue: 1

Pages: 186-200

Print publication date: 13/10/2010

ISSN (print): 0167-4889

ISSN (electronic): 0006-3002



DOI: 10.1016/j.bbamcr.2010.10.002