The p53-inducible small subunit of ribonucleotide reductase (p53R2) was originally assumed to be involved in DNA repair. A new study finds mutations in the gene encoding p53R2 (RRM2B) in individuals with severe mitochondrial DNA depletion in muscle, demonstrating its central role in mitochondrial DNA synthesis.
References
Nordlund, P. & Reichard, P. Annu. Rev. Biochem. 75, 681–706 (2006).
Chabes, A.L., Pfleger, C.M., Kirschner, M.W. & Thelander, L. Proc. Natl. Acad. Sci. USA 100, 3925–3929 (2003).
Tanaka, H. et al. Nature 404, 42–49 (2000).
Nakano, K., Bálint, É., Ashcroft, M. & Vousden, K.H. Oncogene 19, 4283–4289 (2000).
Kimura, T. et al. Nat. Genet. 34, 440–445 (2003).
Powell, D.R. et al. Pediatr. Nephrol. 20, 432–440 (2005).
Bourdon, A. et al. Nat. Genet. 39, 776–780 (2007).
Håkansson, P., Hofer, A. & Thelander, L. J. Biol. Chem. 281, 7834–7841 (2006).
Pontarin, G., Gallinaro, L., Ferraro, P., Reichard, P. & Bianchi, V. Proc. Natl. Acad. Sci. USA 100, 12159–12164 (2003).
Mandel, H. et al. Nat. Genet. 29, 337–341 (2001).
Saada, A. et al. Nat. Genet. 29, 342–344 (2001).
Guittet, O. et al. J. Biol. Chem. 276, 40647–40651 (2001).
Pontarin, G. et al. J. Biol. Chem. 282, published online 7 April 2007 (doi:10.1074/jbc.M701310200).
Engström, Y. & Rozell, B. EMBO J. 7, 1615–1620 (1988).
Chabes, A. et al. Cell 112, 391–401 (2003).
Author information
Authors and Affiliations
Ethics declarations
Competing interests
The author declares no competing financial interests.
Rights and permissions
About this article
Cite this article
Thelander, L. Ribonucleotide reductase and mitochondrial DNA synthesis. Nat Genet 39, 703–704 (2007). https://doi.org/10.1038/ng0607-703
Issue Date:
DOI: https://doi.org/10.1038/ng0607-703
- Springer Nature America, Inc.
This article is cited by
-
Ribonucleotide reductase: In-vitro S-glutathionylation of R2 and p53R2 subunits of mammalian class I ribonucleotide reductase protein
Molecular Biology Reports (2021)
-
Rrm2b deletion causes mitochondrial metabolic defects in renal tubules
Scientific Reports (2019)
-
Mitochondrial Lon sequesters and stabilizes p53 in the matrix to restrain apoptosis under oxidative stress via its chaperone activity
Cell Death & Disease (2018)
-
Pharmacology of natural radioprotectors
Archives of Pharmacal Research (2018)
-
RRM2B Suppresses Activation of the Oxidative Stress Pathway and is Up-regulated by P53 During Senescence
Scientific Reports (2012)