p53R2 Proteins, Antibodies, cDNA Clones, ELISA Kits Research Reagents

RRM2B (Ribonucleotide Reductase Regulatory TP53 Inducible Subunit M2B) is a protein coding gene located on human chromosome 8q22.3. RRM2B is also known as P53R2, MTDPS8A and MTDPS8B. The human RRM2B gene encodes a 40737 Da protein containing 351 amino acids. The RRM2B protein is ubiquitously expressed in thyroid, adrenal and other tissues. Among its related pathways are ATP/ITP metabolism and superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis. RRM2B is related to ribonucleoside-diphosphate reductase activity, thioredoxin disulfide as acceptor. RRM2 is an important paralog of RRM2B gene. RRM2B is associated with some diseases, including Mitochondrial Dna Depletion Syndrome 8A and Progressive External Ophthalmoplegia With Mitochondrial Dna Deletions, Autosomal Dominant 5.

p53R2 Protein (1)

    p53R2 Antibody (7)

      p53R2 ELISA 试剂盒(即用型)& ELISA 抗体对套装(非即用型)(1)

      p53R2 cDNA Clone (19)

      NM_001172477.1

      In expression vector

      NM_199476.1

      克隆载体 cDNA 产品

      In lentiviral vector

      NM_001130543.1

      克隆载体 cDNA 产品

      In lentiviral vector

      p53R2 分子背景

      Ribonucleoside reductase subunit M2B, also known as RRM2B or p53R2, is an enzyme belonging to the iron-dependent ribonucleotide reductase (RNR) enzyme family which is essential for DNA synthesis. Ribonucleotide reductase (RNR) is an enzyme that catalyzes the formation of deoxyribonucleotides from ribonucleotides and plays a critical role in regulating the total rate of DNA synthesis so that DNA to cell mass is maintained at a constant ratio during cell division and DNA repair. RRM2B is a phosphorylated protein. It is hypothesized that RRM2B activity can be regulated at the posttranslational level in response to DNA damage. RRM2B has previously been shown to be essential for the maintenance of mtDNA copy number and its candidacy for tumor suppression has been evaluated in several mutational analyses of different cancer types. However, the contribution of RRM2B to the DNA damage response has been questioned because its transcriptional induction upon DNA damage is not rapid enough for prompt DNA repair. Instead, ATM-mediated phosphorylation has been suggested to regulate the DNA repair activity of RRM2B posttranslationally. Besides, a defect in RRM2B can induce a mild muscle disease of adult onset through disturbance of mitochondrial homeostasis but that this defect does not appear to be oncogenic.

      p53R2 参考文献

      • Bourdon A, et al. (2007) Mutation of RRM2B, encoding p53-controlled ribonucleotide reductase (p53R2), causes severe mitochondrial DNA depletion. Nature Genetics. 39: 776-80.
      • Tyynismaa H, et al. (2009) A Heterozygous Truncating Mutation in RRM2B Causes Autosomal-Dominant Progressive External Ophthalmoplegia with Multiple mtDNA Deletions. AJHG. 85 (2) : 290-5.
      • Shaibani A, et al. (2009) Mitochondrial neurogastrointestinal encephalopathy due to mutations in RRM2B. Arch Neurol.66 (8): 1028-32.

      Note: Flag® is a registered trademark of Sigma Aldrich Biotechnology LP. It is used here for informational purposes only.