Home > Product > Antibody > Rabbit Anti-p53 BP3/FITC Conjugated antibody
AW105885; E3 ubiquitin protein ligase Topors; E3 ubiquitin-protein ligase Topors; LUN; p53 binding protein 3; p53 BP3/LUN; p53-binding protein 3; p53BP3; p53BP3/LUN; Retinitis pigmentosa 31; RP31; SUMO1 protein E3 ligase Topors; SUMO1-protein
Cat:
SL21069R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit,Sheep,)
Immunogen:
KLH conjugated synthetic peptide derived from human p53 BP3
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
119kDa
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Unit:
Price: $
Product PDFs
Datasheet:


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:
Functions as an E3 ubiquitin-protein ligase and as an E3 SUMO1-protein ligase. Probable tumor suppressor involved in cell growth, cell proliferation and apoptosis that regulates p53/TP53 stability through ubiquitin-dependent degradation. May regulate chromatin modification through sumoylation of several chromatin modification-associated proteins. May be involved in DNA damage-induced cell death through IKBKE sumoylation.

Subcellular Location:
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 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.

Post-translational modifications:
Phosphorylation at Ser-98 regulates the E3 ubiquitin-protein ligase activity but not the SUMO1-protein ligase activity. Phosphorylation at Ser-718 increases the E3 ubiquitin-protein ligase activity versus the SUMO1-protein ligase activity resulting in increased p53/TP53 ubiquitination and degradation.
Sumoylated.

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.

Similarity:
Contains 1 RING-type zinc finger.

Database links:

Entrez Gene: 10210 Human

Omim: 609507 Human

SwissProt: Q9NS56 Human

Unigene: 589962 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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