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ribonucleoside-diphosphate reductase subunit M2 B; TP53-inducible ribonucleotide reductase M2 B; p53-inducible ribonucleotide reductase small subunit 2-like protein; p53R2 (RRM2B, P53R2)
Function:
- role in cell survival by repairing damaged DNA (p53-dependent)
- supplies deoxyribonucleotides for DNA repair in cells arrested at G1 or G2
- forms an active ribonucleotide reductase (RNR) complex with RRM1 which is expressed both in resting & proliferating cells in response to DNA damage
- interacts with p53/TP53
- interacts with RRM1 in response to DNA damage
- genetic information processing; DNA replication
2'-deoxyribonucleoside diphosphate + thioredoxin disulfide + H2O
ribonucleoside diphosphate + thioredoxin
Cofactor: binds 2 iron ions per subunit (putative)
Structure:
- heterotetramer with large (RRM1) subunit
- contains an iron-tyrosyl free radical center required for catalysis
- belongs to the ribonucleoside diphosphate reductase small chain family
Compartment:
- cytoplasm, nucleus
- translocates from cytoplasm to nucleus in response to DNA damage
Alternative splicing: named isoforms=5
Expression:
- widely expressed
- high level in skeletal muscle
- weak level in thymus
- expressed in response to DNA damage (p53-dependent)
Pathology:
- defects in RRM2B are the cause of encephalomyopathic mitochondrial depletion syndrome with renal tubulopathy
- expressed in epithelial dysplasias & squamous cell carcinoma
Related
p53R2 gene
General
oxidoreductase
Properties
SIZE: entity length = 351 aa
MW = 41 kD
COMPARTMENT: cytoplasm
cell nucleus
MOTIF: Iron [Fe]-binding site
SITE: 100-100
Iron [Fe]-binding site
SITE: 131-131
Iron [Fe]-binding site
SITE: 134-134
tyrosine residue {Y138}
active site
Iron [Fe]-binding site
SITE: 194-194
Iron [Fe]-binding site
SITE: 228-228
Iron [Fe]-binding site
SITE: 231-231
Database Correlations
OMIM correlations
MORBIDMAP 604712
UniProt Q7LG56
Pfam PF00268
Entrez Gene 50484
Kegg hsa:50484
ENZYME 1.17.4.1
References
- UniProt :accession Q7LG56
- NIEHS-SNPs
http://egp.gs.washington.edu/data/rrm2b/
- Tanaka H, Arakawa H, Yamaguchi T, Shiraishi K, Fukuda S,
Matsui K, Takei Y, Nakamura Y.
A ribonucleotide reductase gene involved in a p53-dependent
cell-cycle checkpoint for DNA damage
Nature. 2000 Mar 2;404(6773):42-9.
PMID: 10716435