Article
Mammalian ribonucleotide reductase subunit p53R2 is required for mitochondrial DNA replication and DNA repair in quiescent cells.
Proceedings of the National Academy of Sciences of the United States of America - 14 Aug 2012
Pontarin Giovanna, Ferraro Paola, Bee Leonardo, Reichard Peter, Bianchi Vera
Abstract excerpt
In postmitotic mammalian cells, protein p53R2 substitutes for protein R2 as a subunit of ribonucleotide reductase. In human patients with mutations in RRM2B, the gene for p53R2, mitochondrial (mt) DNA synthesis is defective, and skeletal muscle presents severe mtDNA depletion. Skin fibroblasts isolated from a patient with a lethal homozygous missense mutation of p53R2 grow normally in culture with an unchanged...
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