Notch 2 functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination. Upon ligand activation through the released notch intracellular domain (NICD) it forms a transcriptional activator complex with RBP-J kappa and activates genes of the enhancer of split locus. Notch 2 is cleaved in the trans-Golgi network, and presented on the cell surface as a heterodimer. It is expressed in the brain, heart, kidney, lung, skeletal muscle and liver. Defects in Nothch 2 are the cause of Alagille syndrome type 2 which is a multisystem disorder defined by hepatic bile duct paucity and cholestasis in association with cardiac, skeletal and opthalmologic manifestations.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.