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MilliporeSigma™ Rat anti-Notch 3, Clone: 2G8,
Rat Monoclonal Antibody
Supplier: MilliporeSigma™ MABF937
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.
Specifications
Notch 3 | |
Monoclonal | |
Purified rat monoclonal IgG2aκ antibody in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% sodium azide. | |
NOTCH3; CASIL; CADASIL; IMF2 | |
KLH-conjugated linear peptide corresponding to a sequence from the EGF-like domains 33-34 region of human Notch 3 ECD. | |
100 μg | |
Inflammation and Immunology | |
NP_000426 | |
Stable for one year at 2°C to 8°C from date of receipt. | |
IgG2a κ |
Immunofluorescence, Immunohistochemistry, Western Blot | |
2G8 | |
Q9UM47 | |
Rat | |
Protein G Purified | |
RUO | |
Primary | |
Human | |
Purified |
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