NOTCH2/3 Antibodies

Target Information

Neurogenic locus notch homolog protein 2 (NOTCH2) is a highly conserved Notch signaling pathway protein. NOTCH2 is a type 1 transmembrane protein with an extracellular domain consisting of multiple epidermal growth factor-like (EGF) repeats, and an intracellular domain consisting of multiple, different domain types. NOTCH2 functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination. Defects in NOTCH2 are the cause of Alagille syndrome type 2 (ALGS2). Alagille syndrome is an autosomal dominant multisystem disorder defined clinically by hepatic bile duct paucity and cholestasis in association with cardiac, skeletal, and ophthalmologic manifestations. There are characteristic facial features and less frequent clinical involvement of the renal and vascular systems.

NOTCH3 is the third discovered human homologue of the Drosophila melanogaster type I membrane protein notch. In Drosophila, notch interaction (with its cell-bound ligands delta, serrate) establishes an intercellular signaling pathway that plays a key role in neural development. Homologues of the notch-ligands have also been identified in human. NOTCH3 functions as a receptor for membrane-bound ligands Jagged1, Jagged2, and Delta1 to regulate cell-fate determination. Mutations in NOTCH3 have been identified as the underlying cause of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).