基因名称(Gene Name)
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
免疫原
Synthesized peptide derived from part region of human protein
特异性
TOPRS Monoclonal Antibody detects endogenous levels of protein.
组成
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
背景
This gene encodes a nuclear protein which is serine and arginine rich, and contains a RING-type zinc finger domain. It is highly expressed in the testis, and functions as an ubiquitin-protein E3 ligase. Mutations in this gene are associated with retinitis pigmentosa type 31. Alternatively spliced transcript variants, encoding different isoforms, have been observed for this locus. [provided by RefSeq, Sep 2010],
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 o
