功能： caution:Was originally (PubMed:11278651) thought to bind to the palindromic consensus sequence 5'-TCCCAGCACTTTGGGA-3' and to regulate the transcription of numerous genes in the lung.,disease:Defects in TOPORS are the cause of retinitis pigmentosa type 31 (RP31) [MIM:609923]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP31 inheritance is autosomal dominant.,function:Functions as an ubiquitin-protein E3 ligase and as an E3 SUMO1-protein ligase. Can both ubiquitinate and sumoylate p53.,induction:By genotoxic agents such as cisplatin and camptothecin.,PTM:Sumoylated.,similarity:Contains 1 RING-type zinc finger.,subcellular location:Localizes to discrete nuclear foci which partly overlap with PML nuclear bodies.,subunit:Interacts with PARK7/DJ-1 (By similarity). Interacts with TOP1. Interacts with p53. Interacts with the SUMO1 conjugating enzyme UBE2I. Interacts with SUMO1.,tissue specificity:Expressed at highest levels in testis and at lower levels in adrenal gland, bone marrow, brain, colon, heart, kidney, liver, muscle, ovary, pancreas, placenta, prostate, skeletal muscle, skin, small intestine, spleen, stomach, testis, thymus, thyroid and uterus. Expressed in the alveolar epithelium of the lung. Expression is commonly decreased in colon adenocarcinomas and lung cancers.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Nucleus. Nucleus, PML body. Localizes to discrete nuclear foci which partly overlap with PML nuclear bodies. Targeted to PML nuclear bodies upon DNA damage.
组织表达： Expressed at highest levels in testis and at lower levels in adrenal gland, bone marrow, brain, colon, heart, kidney, liver, muscle, ovary, pancreas, placenta, prostate, skeletal muscle, skin, small intestine, spleen, stomach, testis, thymus, thyroid and uterus. Expressed in the alveolar epithelium of the lung. Expression is commonly decreased in colon adenocarcinomas and lung cancers.
科研货号： PLA007557
TOPRS Polyclonal Antibody
Catalog NoPLA007557
Product information
基因名称： TOPORS LUN TP53BPL
蛋白名称： E3 ubiquitin-protein ligase Topors (EC 6.3.2.-) (SUMO1-protein E3 ligase Topors) (Topoisomerase I-binding RING finger protein) (Topoisomerase I-binding arginine/serine-rich protein) (Tumor suppressor p53-binding protein 3) (p53-binding protein 3) (p53BP3)
功能： caution:Was originally (PubMed:11278651) thought to bind to the palindromic consensus sequence 5'-TCCCAGCACTTTGGGA-3' and to regulate the transcription of numerous genes in the lung.,disease:Defects in TOPORS are the cause of retinitis pigmentosa type 31 (RP31) [MIM:609923]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP31 inheritance is autosomal dominant.,function:Functions as an ubiquitin-protein E3 ligase and as an E3 SUMO1-protein ligase. Can both ubiquitinate and sumoylate p53.,induction:By genotoxic agents such as cisplatin and camptothecin.,PTM:Sumoylated.,similarity:Contains 1 RING-type zinc finger.,subcellular location:Localizes to discrete nuclear foci which partly overlap with PML nuclear bodies.,subunit:Interacts with PARK7/DJ-1 (By similarity). Interacts with TOP1. Interacts with p53. Interacts with the SUMO1 conjugating enzyme UBE2I. Interacts with SUMO1.,tissue specificity:Expressed at highest levels in testis and at lower levels in adrenal gland, bone marrow, brain, colon, heart, kidney, liver, muscle, ovary, pancreas, placenta, prostate, skeletal muscle, skin, small intestine, spleen, stomach, testis, thymus, thyroid and uterus. Expressed in the alveolar epithelium of the lung. Expression is commonly decreased in colon adenocarcinomas and lung cancers.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Nucleus. Nucleus, PML body. Localizes to discrete nuclear foci which partly overlap with PML nuclear bodies. Targeted to PML nuclear bodies upon DNA damage.
组织表达： Expressed at highest levels in testis and at lower levels in adrenal gland, bone marrow, brain, colon, heart, kidney, liver, muscle, ovary, pancreas, placenta, prostate, skeletal muscle, skin, small intestine, spleen, stomach, testis, thymus, thyroid and uterus. Expressed in the alveolar epithelium of the lung. Expression is commonly decreased in colon adenocarcinomas and lung cancers.
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