Hamster Anti-NOTCH3 Recombinant Antibody (CBWJN-0609) (CBMAB-N0474-WJ)

Name: Hamster Anti-NOTCH3 Recombinant Antibody (CBWJN-0609)
SKU: CBMAB-N0474-WJ
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Hamster

Clone

CBWJN-0609

Application

Specificity

Human

Antibody Isotype

IgG

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!]

Format

Liquid

Concentration

1.0 mg/mL

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

Notch 3

Introduction

This gene encodes the third discovered human homologue of the Drosophilia melanogaster type I membrane protein notch. In Drosophilia, notch interaction with its cell-bound ligands (delta, serrate) establishes an intercellular signalling pathway that plays a key role in neural development. Homologues of the notch-ligands have also been identified in human, but precise interactions between these ligands and the human notch homologues remains to be determined. Mutations in NOTCH3 have been identified as the underlying cause of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). [provided by RefSeq, Jul 2008]

Entrez Gene ID

4854

UniProt ID

Q9UM47

Alternative Names

Notch 3; Neurogenic Locus Notch Homolog Protein 3; Notch (Drosophila) Homolog 3; Notch Homolog 3 (Drosophila); Notch Homolog 3; CADASIL1;

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.

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
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For research use only. Not intended for any clinical use.

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