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Rat Anti-NOTCH3 Recombinant Antibody (NIZ 8G5) (CBMAB-0530CQ)


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Summary

Host Animal
Rat
Specificity
Human
Clone
NIZ 8G5
Antibody Isotype
IgG2a
Application
WB

Basic Information

Immunogen
Recombinant full length protein from Human NOTCH3 aa 1-2321
Specificity
Human
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Notch 3
Introduction
This gene encodes the third discovered human homologue of the Drosophilia melanogaster type I membrane protein notch. The NOTCH3 gene encodes a single pass transmembrane protein belonging to an evolutionarily conserved NOTCH receptor family (see, e.g., NOTCH1; 190198). After ligand binding, the intracellular domain translocates to the nucleus and activates transcription factors. The Notch signaling pathway plays a central role in the development and maturation of most vertebrate organs, with pleiotropic effects depending on dose and context. Mutations in NOTCH3 have been identified as the underlying cause of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
Entrez Gene ID
4854
UniProt ID
Q9UM47
Alternative Names
NOTCH3; notch 3; IMF2; CASIL; CADASIL; neurogenic locus notch homolog protein 3; Notch homolog 3
Function
Functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination (PubMed:15350543).
Upon ligand activation through the released notch intracellular domain (NICD) it forms a transcriptional activator complex with RBPJ/RBPSUH and activates genes of the enhancer of split locus. Affects the implementation of differentiation, proliferation and apoptotic programs (By similarity).
Biological Process
Artery morphogenesisIEA:Ensembl
Axon guidanceManual Assertion Based On ExperimentIBA:GO_Central
Forebrain developmentIEA:Ensembl
Glomerular capillary formationIEA:Ensembl
Negative regulation of neuron differentiationIEA:Ensembl
Negative regulation of transcription by RNA polymerase IIIEA:Ensembl
Neuron fate commitmentIEA:Ensembl
Notch signaling pathwayManual Assertion Based On ExperimentIBA:GO_Central
Positive regulation of smooth muscle cell proliferationIEA:Ensembl
Positive regulation of transcription by RNA polymerase IIIEA:Ensembl
Cellular Location
Cell membrane
Notch 3 intracellular domain
Nucleus
Following proteolytical processing NICD is translocated to the nucleus.
Involvement in disease
Cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy, 1 (CADASIL1):
A cerebrovascular disease characterized by multiple subcortical infarcts, pseudobulbar palsy, dementia, and the presence of granular deposits in small cerebral arteries producing ischemic stroke.
Myofibromatosis, infantile 2 (IMF2):
A rare mesenchymal disorder characterized by the development of benign tumors in the skin, striated muscles, bones, and, more rarely, visceral organs. Subcutaneous or soft tissue nodules commonly involve the skin of the head, neck, and trunk. Skeletal and muscular lesions occur in about half of the patients. Lesions may be solitary or multicentric, and they may be present at birth or become apparent in early infancy or occasionally in adult life. Visceral lesions are associated with high morbidity and mortality.
Lateral meningocele syndrome (LMNS):
A very rare skeletal disorder with facial anomalies, hypotonia and neurologic dysfunction due to meningocele, a protrusion of the meninges, unaccompanied by neural tissue, through a bony defect in the skull or vertebral column. LMNS facial features include hypertelorism and telecanthus, high arched eyebrows, ptosis, mid-facial hypoplasia, micrognathia, high and narrow palate, low-set ears and a hypotonic appearance. Additional variable features are connective tissue abnormalities, aortic dilation, a high-pitched nasal voice, wormian bones and osteolysis.
Topology
Extracellular: 40-1643
Helical: 1644-1664
Cytoplasmic: 1665-2321
PTM
Synthesized in the endoplasmic reticulum as an inactive form which is proteolytically cleaved by a furin-like convertase in the trans-Golgi network before it reaches the plasma membrane to yield an active, ligand-accessible form. Cleavage results in a C-terminal fragment N(TM) and a N-terminal fragment N(EC). Following ligand binding, it is cleaved by TNF-alpha converting enzyme (TACE) to yield a membrane-associated intermediate fragment called notch extracellular truncation (NEXT). This fragment is then cleaved by presenilin dependent gamma-secretase to release a notch-derived peptide containing the intracellular domain (NICD) from the membrane.
Phosphorylated.
Hydroxylated by HIF1AN.
More Infomation

Zhang, Y., Hernandez, M., Gower, J., Winicki, N., Morataya, X., Alvarez, S., ... & Thistlethwaite, P. A. (2022). JAGGED-NOTCH3 signaling in vascular remodeling in pulmonary arterial hypertension. Science translational medicine, 14(643), eabl5471.

Gravesteijn, G., Hack, R. J., Mulder, A. A., Cerfontaine, M. N., van Doorn, R., Hegeman, I. M., ... & Lesnik Oberstein, S. A. (2022). NOTCH3 variant position is associated with NOTCH3 aggregation load in CADASIL vasculature. Neuropathology and Applied Neurobiology, 48(1), e12751.

Schoemaker, D., & Arboleda-Velasquez, J. F. (2021). Notch3 signaling and aggregation as targets for the treatment of CADASIL and other NOTCH3-associated small-vessel diseases. The American Journal of Pathology, 191(11), 1856-1870.

Cho, B. P., Nannoni, S., Harshfield, E. L., Tozer, D., Gräf, S., Bell, S., & Markus, H. S. (2021). NOTCH3 variants are more common than expected in the general population and associated with stroke and vascular dementia: an analysis of 200 000 participants. Journal of Neurology, Neurosurgery & Psychiatry, 92(7), 694-701.

Xiu, M., Wang, Y., Li, B., Wang, X., Xiao, F., Chen, S., ... & Hua, F. (2021). The role of Notch3 signaling in cancer stemness and chemoresistance: molecular mechanisms and targeting strategies. Frontiers in Molecular Biosciences, 8, 694141.

Zhang, Y. Q., Liang, Y. K., Wu, Y., Chen, M., Chen, W. L., Li, R. H., ... & Zhang, G. J. (2021). Notch3 inhibits cell proliferation and tumorigenesis and predicts better prognosis in breast cancer through transactivating PTEN. Cell Death & Disease, 12(6), 502.

Hosseini-Alghaderi, S., & Baron, M. (2020). Notch3 in development, health and disease. Biomolecules, 10(3), 485.

Papakonstantinou, E., Bacopoulou, F., Brouzas, D., Megalooikonomou, V., D’Elia, D., Bongcam-Rudloff, E., & Vlachakis, D. (2019). NOTCH3 and CADASIL syndrome: a genetic and structural overview. EMBnet. journal, 24.

Morris, H. E., Neves, K. B., Montezano, A. C., MacLean, M. R., & Touyz, R. M. (2019). Notch3 signalling and vascular remodelling in pulmonary arterial hypertension. Clinical Science, 133(24), 2481-2498.

Rutten, J. W., Van Eijsden, B. J., Duering, M., Jouvent, E., Opherk, C., Pantoni, L., ... & Lesnik Oberstein, S. A. (2019). The effect of NOTCH3 pathogenic variant position on CADASIL disease severity: NOTCH3 EGFr 1–6 pathogenic variant are associated with a more severe phenotype and lower survival compared with EGFr 7–34 pathogenic variant. Genetics in Medicine, 21(3), 676-682.

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For research use only. Not intended for any clinical use.

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