Anti-NOTCH3 antibody [4HCLC]

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

The NOTCH3 protein a significant player in the NOTCH signaling pathway is a transmembrane receptor involved in cell differentiation. Sometimes referred to as 'Notch3' this protein has a molecular weight of approximately 244 kDa. NOTCH3 is widely expressed in vascular smooth muscle cells and tissues such as arteries and lungs. The protein also plays an important role in the communication between adjacent cells because of its location on the cell surface.

Biological function summary

NOTCH3 influences various cellular processes including proliferation apoptosis and differentiation. NOTCH3 does not function alone it is part of a regulatory complex that mediates cellular context and fate decisions. The receptor is cleaved upon ligand binding to facilitate intracellular signaling. This signaling is critical in the development and maintenance of vascular integrity which highlights the protein's importance in both physiological and pathological contexts.

Pathways

NOTCH3 plays a role in the NOTCH signaling pathway essential for cell fate determination and the smooth muscle cell differentiation pathway. Ligands such as Jagged and Delta-like proteins trigger NOTCH3 activation. Upon activation the NOTCH3 intracellular domain translocates to the nucleus where it interacts with transcriptional regulators like RBPJ. This interaction modulates the expression of target genes influencing cell differentiation and proliferation.

Associated diseases and disorders

NOTCH3 is implicated in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) a genetic vascular disorder. Mutations in the NOTCH3 gene result in the accumulation of the NOTCH3 protein within arterial walls leading to the characteristic symptoms of CADASIL. Additionally the protein has been linked to pulmonary arterial hypertension through shared pathways with proteins such as BMP2 and SMADs indicating its broader impact on vascular pathophysiology.