Mouse anti-Human Notch3 Monoclonal Antibody | anti-NOTCH3 antibody

Notch3, CT (Notch homolog 3, Drosophila, hN3, CASIL, CADASIL)

Cat. Num. AAA649504

• Gene Names: NOTCH3; IMF2; CASIL; CADASIL
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein G affinity chromatography.
• Synonyms: Notch3; Monoclonal Antibody; CT (Notch homolog 3; Drosophila; hN3; CASIL; CADASIL); Anti -Notch3; anti-NOTCH3 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 487CT6.9.2
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein G affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.1% sodium azide.

• Applicable Applications for anti-NOTCH3 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in Western Blot, ELISA.; Dilution: ELISA: 1:1,000; Western Blot: 1:100~250
• Immunogen: NOTCH3 Mab is generated from mouses immunized with a KLH conjugated synthetic peptide selected from the C-term region of human NOTCH3.
• Preparation and Storage: May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Western Blot (WB)

(NOTCH3 Antibody (Q2306) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the NOTCH3 antibody detected the NOTCH3 protein (arrow).)

Related Product Information for anti-NOTCH3 antibody

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].

Product Categories/Family for anti-NOTCH3 antibody

Antibodies; Abs to Notch Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 134244285
• NCBI GeneID: 4854
• NCBI Accession #: NP_000426.2
• NCBI GenBank Nucleotide #: NM_000435.2
• UniProt Accession #: Q9UM47; Q9UEB3; Q9UPL3; Q9Y6L8
• Molecular Weight: 243,631 Da
• NCBI Official Full Name: neurogenic locus notch homolog protein 3
• NCBI Official Synonym Full Names: notch 3
• NCBI Official Symbol: NOTCH3
• NCBI Official Synonym Symbols: IMF2; CASIL; CADASIL
• NCBI Protein Information: neurogenic locus notch homolog protein 3; Notch homolog 3
• UniProt Protein Name: Neurogenic locus notch homolog protein 3
• Protein Family: Neurogenic locus notch homolog protein
• UniProt Gene Name: NOTCH3
• UniProt Synonym Gene Names: Notch 3
• UniProt Entry Name: NOTC3_HUMAN

NCBI Description

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]

Uniprot Description

Function: Functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination. 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.

Subunit structure: Heterodimer of a C-terminal fragment N(TM) and a N-terminal fragment N(EC) which are probably linked by disulfide bonds

By similarity. Interacts with MAML1, MAML2 and MAML3 which act as transcriptional coactivators for NOTCH3. Interacts with PSMA1. Interacts with HIF1AN. Ref.5 Ref.6 Ref.7 Ref.8

Subcellular location: Cell membrane; Single-pass type I membrane protein. Notch 3 intracellular domain: Nucleus. Note: Following proteolytical processing NICD is translocated to the nucleus.

Tissue specificity: Ubiquitously expressed in fetal and adult tissues.

Post-translational modification: 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

By similarity.Phosphorylated

By similarity.Hydroxylated by HIF1AN.

Involvement in disease: Cerebral arteriopathy with subcortical infarcts and leukoencephalopathy, autosomal dominant (CADASIL) [MIM:125310]: A cerebrovascular disease characterized by multiple subcortical infarcts, pseudobulbar palsy, dementia, and the presence of granular deposits in small cerebral arteries producing ischemic stroke.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10 Ref.11 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30Myofibromatosis, infantile 2 (IMF2) [MIM:615293]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.30 Ref.31

Sequence similarities: Belongs to the NOTCH family.Contains 5 ANK repeats.Contains 34 EGF-like domains.Contains 3 LNR (Lin/Notch) repeats.