RBPJ : recombination signal binding protein for immunoglobulin kappa J region


Description

The RBPJ (recombination signal binding protein for immunoglobulin kappa J region) is a protein-coding gene located on chromosome 4.

The RBPJ gene provides instructions for making a protein called RBP-J, which is a key component of the Notch signaling pathway. Notch signaling governs the development of specific cell types during embryonic growth, including those that form bones, heart, muscles, nerves, and blood. When the Notch pathway is activated, the RBP-J protein binds to particular regions of DNA, controlling the activity of genes involved in cell development.

RBPJ is a transcriptional regulator that plays a crucial role in Notch signaling, a communication pathway between cells that influences cell development. It acts as a transcriptional repressor in the absence of Notch proteins, but when associated with Notch intracellular domain (NICD), it becomes a transcriptional activator, turning on Notch target genes. It likely controls gene expression by recruiting chromatin remodeling complexes containing histone deacetylase or histone acetylase. RBPJ has a specific binding affinity for immunoglobulin kappa-type J segment recombination signal sequence and methylated DNA. It also binds to the oxygen responsive element of COX4I2, activating its transcription under hypoxic conditions. RBPJ negatively regulates the phagocyte oxidative burst response to bacterial infection by repressing the transcription of NADPH oxidase subunits.

RBPJ is also known as AOS3, CBF-1, CBF1, IGKJRB, IGKJRB1, KBF2, RBP-J, RBP-J kappa, RBP-JK, RBPJK, RBPSUH, SUH, csl.

Associated Diseases


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