功能： 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. Forms an active ribonucleotide reductase (RNR) complex with RRM1 which is expressed both in resting and proliferating cells in response to DNA damage.,induction:In response to DNA damage in a wild-type p53/TP53-dependent manner.,pathway:Genetic information processing; DNA replication.,similarity:Belongs to the ribonucleoside diphosphate reductase small chain family.,subcellular location:Translocates from cytoplasm to nucleus in response to DNA damage.,subunit:Heterotetramer with large (RRM1) subunit. Interacts with p53/TP53. Interacts with RRM1 in response to DNA damage.,tissue specificity:Widely expressed at a high level in skeletal muscle and at a weak level in thymus. Expressed in epithelial dysplasias and squamous cell carcinoma.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cytoplasm. Nucleus. Translocates from cytoplasm to nucleus in response to DNA damage.
组织表达： Widely expressed at a high level in skeletal muscle and at a weak level in thymus. Expressed in epithelial dysplasias and squamous cell carcinoma.
科研货号： PLA017479
p53R2 Polyclonal Antibody
Catalog NoPLA017479
Product information
基因名称： RRM2B
蛋白名称： Ribonucleoside-diphosphate reductase subunit M2 B
功能： 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. Forms an active ribonucleotide reductase (RNR) complex with RRM1 which is expressed both in resting and proliferating cells in response to DNA damage.,induction:In response to DNA damage in a wild-type p53/TP53-dependent manner.,pathway:Genetic information processing; DNA replication.,similarity:Belongs to the ribonucleoside diphosphate reductase small chain family.,subcellular location:Translocates from cytoplasm to nucleus in response to DNA damage.,subunit:Heterotetramer with large (RRM1) subunit. Interacts with p53/TP53. Interacts with RRM1 in response to DNA damage.,tissue specificity:Widely expressed at a high level in skeletal muscle and at a weak level in thymus. Expressed in epithelial dysplasias and squamous cell carcinoma.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cytoplasm. Nucleus. Translocates from cytoplasm to nucleus in response to DNA damage.
组织表达： Widely expressed at a high level in skeletal muscle and at a weak level in thymus. Expressed in epithelial dysplasias and squamous cell carcinoma.
在线咨询
湘公网安备