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Anti-RBPJ Antibody

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产品名称: Anti-RBPJ Antibody
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Anti-RBPJ Antibody


Anti-RBPJ Antibody  的详细介绍
Name: Anti-RBPJ Antibody
See all RBPJ primary antibodies
Description: Rabbit polyclonal antibody to RBPJ
Specificity: The antibody detects endogenous level of total RBPJ protein.
Applications: WB, IHC
Reactivity: Human, Mouse, Rat
Immunogen: Recombinant protein of human RBPJ.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Antibodies were purified by affinity purification using immunogen.
Concentration: 1.0mg / ml
Formulation: Supplied at 1.0mg / mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage: Store at -20?C
Function: Transcriptional regulator that plays a central role in Notch signaling, a signaling pathway involved in cell-cell communication that regulates a broad spectrum of cell-fate determinations. Acts as a transcriptional repressor when it is not associated with Notch proteins. When associated with some NICD product of Notch proteins (Notch intracellular domain), it acts as a transcriptional activator that activates transcription of Notch target genes. Probably represses or activates transcription via the recruitment of chromatin remodeling complexes containing histone deacetylase or histone acetylase proteins, respectively. Specifically binds to the immunoglobulin kappa-type J segment recombination signal sequence. Binds specifically to methylated DNA. Binds to the oxygen responsive element of COX4I2 and activates its transcription under hypoxia conditions (4% oxygen) (PubMed:23303788).
Involvement in Disease: Adams-Oliver syndrome 3: An autosomal dominant form of Adams-Oliver syndrome, a disorder characterized by the congenital absence of skin (aplasia cutis congenita) in combination with transverse limb defects. Aplasia cutis congenita can be located anywhere on the body, but in the vast majority of the cases, it is present on the posterior parietal region where it is often associated with an underlying defect of the parietal bones. Limb abnormalities are typically limb truncation defects affecting the distal phalanges or entire digits (true ectrodactyly). Only rarely, metatarsals/metacarpals or more proximal limb structures are also affected. Apart from transverse limb defects, syndactyly, most commonly of second and third toes, can also be observed. The clinical features are highly variable and can also include cardiovascular malformations, brain abnormalities and vascular defects such as cutis marmorata and dilated scalp veins. AOS3 patients manifest characteristic vertex scalp defects and terminal limb defects, but without congenital heart defects, other associated defects, or immune defects.
Sequence Similarities: Belongs to the Su(H) family.
Cellular Location: Nucleus. Cytoplasm.

Mainly nuclear, upon interaction with RITA/C12orf52, translocates to the cytoplasm, down-regulating the Notch signaling pathway.
Database Links:
  • Entrez Gene: 3516?Human
  • Entrez Gene: 19664?Mouse
  • Entrez Gene: 679028?Rat
  • Omim: 147183?Human
  • SwissProt: Q06330?Human
  • SwissProt: P31266?Mouse
  • Unigene: 479396?Human
  • Unigene: 209292?Mouse
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    Synonyms:
  • AI843960 Antibody
  • AOS3 Antibody
  • CBF 1 Antibody
  • CBF-1 Antibody
  • CBF1 Antibody
  • csl Antibody
  • IGKJRB Antibody
  • IGKJRB1 Antibody
  • J kappa recombination signal binding protein Antibody
  • J kappa-recombination signal-binding protein Antibody
  • KBF2 Antibody
  • NY REN 30 antigen Antibody
  • RBP J Antibody
  • RBP J kappa Antibody
  • RBP JK Antibody
  • RBP-J Antibody
  • RBP-J kappa Antibody
  • RBP-JK Antibody
  • Rbpj Antibody
  • RBPJK Antibody
  • RBPSUH Antibody
  • recombination signal binding protein for immunoglobulin kappa J region Antibody
  • Recombining binding protein suppressor of hairless Antibody
  • Renal carcinoma antigen NY-REN-30 Antibody
  • SUH Antibody
  • SUH_HUMAN Antibody
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    Information: Target information shown above is from the UniProt Consortium.
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