MilliporeSigma™ Rat anti-Notch 3, Clone: 2G8,

Description

• Specifically detects Notch 3 clone: 2G8 in Human samples, and it is validated for Immunofluorescence, Immunohistochemistry, Western Blotting Neurogenic locus notch homolog protein 3 (UniProt Q9UM47; also known as Notch 3, Notch homolog 3) is encoded by the NOTCH3 (also known as CASIL, CADASIL, IMF2) gene (Gene ID 4854) in human
• Notch 3 is a transmembrane receptor required for arterial differentiation and maturation of vascular smooth muscle cells in small arteries
• Notch 3 is initially produced with a signal peptide (a.a
• 1-39), the removal of which yields the 2282-amino acid Notch 3 (a.a
• 40-2321), which undergoes a constitutive proteolytical cleavage, generating the extracellular fragment (Notch3-ECD; a.a
• 40-1628) and the transmembrane/cytosolic (Notch3-TMIC; a.a
• 1629-2321) fragment that remain associated at the cell surface to form a heterodimeric receptor
• The ECD fragment contains all 34 EGF-like domains and the three Lin/Notch (LNR) repeats, while the extracellular portion of TMIC contains 5 ankyrin repeats
• Mutations in the NOTCH3 gene have been identified as the underlying cause of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
• Pathogenic mutations predominantly affect cysteine residues within EGF-like repeats of Notch3-ECD, leading to Notch3-ECD multimerization and accumulation in the tunica media of vessel walls.