Molecular weight (DA)
114kD
Immunogen
Synthesized peptide derived from part region of human protein
Specificity
TOPRS Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
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],
Function
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
Gene Name
TOPORS LUN TP53BPL
Protein name
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
Human protein sequence Database
Q9NS56
Mouse protein sequence database
Q80Z37
Rat protein sequence database
Cellular localization
Nucleus. Nucleus, PML body. Localizes to discrete nuclear foci which partly overlap with PML nuclear bodies. Targeted to PML nuclear bodies upon DNA damage.
Tissue expression
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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