Recombinant Human NOTCH3

Background

NOTCH3 is a receptor in the Notch signaling pathway that primarily regulates vascular smooth muscle cell differentiation, stability, and proliferation. Ligand binding triggers cleavage and release of the NOTCH3 intracellular domain (NICD), which activates gene transcription to maintain vascular integrity. NOTCH3 is highly expressed in arterial smooth muscle cells, where it supports vessel structure and function. Mutations in NOTCH3 cause CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy), a genetic disorder characterized by recurrent strokes, dementia, and vascular dysfunction due to smooth muscle cell degeneration. NOTCH3 is also implicated in tumor biology, where its dysregulation contributes to cancer cell survival, metastasis, and angiogenesis. Knockout studies show defects in vascular development and smooth muscle cell maintenance, emphasizing its role in vascular homeostasis, cell differentiation, and disease pathology.