Molecular weight (DA)
40kD
Immunogen
Synthesized peptide derived from the Internal region of human p53R2.
Specificity
p53R2 Polyclonal Antibody detects endogenous levels of p53R2 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes the small subunit of a p53-inducible ribonucleotide reductase. This heterotetrameric enzyme catalyzes the conversion of ribonucleoside diphosphates to deoxyribonucleoside diphosphates. The product of this reaction is necessary for DNA synthesis. Mutations in this gene have been associated with autosomal recessive mitochondrial DNA depletion syndrome, autosomal dominant progressive external ophthalmoplegia-5, and mitochondrial neurogastrointestinal encephalopathy. Alternatively spliced transcript variants have been described.[provided by RefSeq, Feb 2010],
Function
catalytic activity:2'-deoxyribonucleoside diphosphate + thioredoxin disulfide + H(2)O = ribonucleoside diphosphate + thioredoxin.,cofactor:Binds 2 iron ions per subunit.,disease:Defects in RRM2B are the cause of encephalomyopathic mitochondrial depletion syndrome with renal tubulopathy (EMDSRT) [MIM:612075]. Mitochondrial DNA depletion syndrome (MDS) is a clinically heterogeneous group of disorders characterized by a reduction in mitochondrial DNA (mtDNA) copy number. The encephalomyopathic form with renal tubulopathy is presented with various combinations of hypotonia, tubulopathy, seizures, respiratory distress, diarrhea, and lactic acidosis.,function:Plays a pivotal role in cell survival by repairing damaged DNA in a p53/TP53-dependent manner. Supplies deoxyribonucleotides for DNA repair in cells arrested at G1 or G2. Contains an iron-tyrosyl free radical center required for catalysis
Protein name
Ribonucleoside-diphosphate reductase subunit M2 B
Other name
RRM2B; P53R2; Ribonucleoside-diphosphate reductase subunit M2 B; TP53-inducible ribonucleotide reductase M2 B; p53-inducible ribonucleotide reductase small subunit 2-like protein; p53R2
Fields
>>Purine metabolism;>>Pyrimidine metabolism;>>Glutathione metabolism;>>Drug metabolism - other enzymes;>>Metabolic pathways;>>Nucleotide metabolism;>>p53 signaling pathway
Human protein sequence Database
Q7LG56
Mouse protein sequence database
Q6PEE3
Rat protein sequence database
Cellular localization
Cytoplasm. Nucleus. Translocates from cytoplasm to nucleus in response to DNA damage.
Tissue expression
Widely expressed at a high level in skeletal muscle and at a weak level in thymus. Expressed in epithelial dysplasias and squamous cell carcinoma.
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