RBPJ: 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. Interacts with activated NOTCH1, NOTCH2 or NOTCH3. Interacts with MINT/SHARP. This interaction may mediate the recruitment of large corepressor complexes containing proteins such as HDAC1, HDAC2, NCOR2, SAP30, FHL1/KYOT2 and CIR1. Interacts with EP300, MAML1 and PTF1A. Interacts with Epstein-Barr virus EBNA2, EBNA3, EBNA4 and EBNA6. Interacts with RITA/C12orf52, leading to nuclear export, prevent the interaction between RBPJ and NICD product and subsequent down-regulation of the Notch signaling pathway. Belongs to the Su(H) family. 7 isoforms of the human protein are produced by alternative splicing.
Protein type: Cell development/differentiation; Transcription factor
Chromosomal Location of Human Ortholog: 4p15.2
Cellular Component: nucleoplasm; transcription factor complex; cytoplasm; nucleolus; nucleus
Molecular Function: protein binding; DNA binding; recombinase activity; protein N-terminus binding; chromatin binding; transcription factor activity
Biological Process: somatic stem cell maintenance; myeloid dendritic cell differentiation; sebaceous gland development; negative regulation of transcription from RNA polymerase II promoter; DNA recombination; negative regulation of cell proliferation; negative regulation of ossification; angiogenesis; regulation of timing of cell differentiation; positive regulation of BMP signaling pathway; positive regulation of cardiac muscle cell proliferation; transcription initiation from RNA polymerase II promoter; epidermal cell fate specification; Notch signaling pathway; hair follicle maturation; humoral immune response; activation of Notch receptor target transcription factor; keratinocyte differentiation; inflammatory response to antigenic stimulus; pituitary gland development; B cell differentiation; defense response to bacterium; epithelial to mesenchymal transition; positive regulation of transcription from RNA polymerase II promoter; blood vessel remodeling; gene expression; negative regulation of transcription, DNA-dependent; auditory receptor cell fate commitment
Disease: Adams-oliver Syndrome 3
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