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MABC594

Sigma-Aldrich

Anti-NOTCH 3/N3ECD Antibody, clone 1E4

clone 1E4, 1 mg/mL, from mouse

Synonyme(s) :

Neurogenic locus notch homolog protein 3, Notch 3, Notch 3 extracellular truncation, Notch 3 intracellular domain, N3ECD Domain, Ectodermal N3ECD, N3ECD

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About This Item

Code UNSPSC :
12352203
eCl@ss :
32160702
Nomenclature NACRES :
NA.41

Source biologique

mouse

Niveau de qualité

Forme d'anticorps

purified immunoglobulin

Type de produit anticorps

primary antibodies

Clone

1E4, monoclonal

Espèces réactives

human

Concentration

1 mg/mL

Technique(s)

electron microscopy: suitable
immunocytochemistry: suitable
western blot: suitable

Isotype

IgG1κ

Numéro d'accès NCBI

Numéro d'accès UniProt

Conditions d'expédition

wet ice

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... NOTCH3(4854)

Description générale

NOTCH3 is a neuronal cell fate regulating protein that initially functions as a receptor for the membrane bound ligands Jagged1, Jagged2 and Delta1. NOTCH3 gene functions operate via the intracellular domain of Notch3 (NICD) that forms a transcriptional activator complex with RBPJ/RBPSUH and activates genes of the enhancer of split locus which ultimately influences cellular differentiation, proliferation and apoptotic programs. NOTCH3 is widely expressed in fetal and adult tissues. NOTCH3 protein begins as an inactive form in the endoplasmic reticulum that becomes processed to an active form in the plasma membrane. Functionally, NOTCH3 is cleaved by TACE to yield a membrane bound extracellular fragment called NEXT which is then cleaved by gamma-secretase to release the NICD intracellular peptide from the membrane, which then proceeds to be translocated to the nucleus for gene the gene activations. Mutations in NOTCH3 lead to cerebral arteriopathy with subcortical infarcts and leukoencephalopathy syndrome and myofibromatosis infantile 2 disorder, both seriously debilitating genetic diseases. Disease-causing mutations affect cysteine residues within epidermal growth factor-like repeat domains in the ectodermal domain of NOTCH3 (N3ECD). One of the main biochemical and histopathological hallmarks of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the accumulation of N3ECD at the cell surface of vascular smooth muscle cells which degenerate over the course of the disease.

Spécificité

This antibody detects both the full length NOTCH 3 protein and the NOTCH 3 ectodermal domain (N3ECD).

Immunogène

His-tagged recombinant protein corresponding to Human NOTCH 3.

Application

Immunocytochemistry Analysis: A representative lot of this antibody was used to detect NOTCH 3 in CADASIL vascular smooth muscle cells (Tikka, S., et al. (2012) Journal of Cerebral Blood Flow & Metabolism. 1–10).

Immunohistochemistry Analysis: A representative lot of this antibody was used to detect NOTCH 3 in Huamn vascular smooth muscle cells & smooth muscle cells of vessels from CADASIL brain tissue (Jouet, A., et al. (2000) Journal of Clinical Investigation. 105(5):597-605).

Electron Microscopy: A representative lot of this antibody was used to detect NOTCH 3 in smooth muscle cells of vessels from CADASIL brain tissue (Jouet, A., et al. (2000) Journal of Clinical Investigation. 105(5):597-605).

Immunohistochemistry Analysis: A representative lot of this antibody was used to detect NOTCH 3 in vessels of a CADASIL patient (Rouchox, M.M., et al. (2003) American Journal of Pathology. 162(1):329-342).

Immunohistochemistry Analysis: A representative lot of this antibody was used to detect NOTCH 3 in Human colorectal carcinoma (Serafin, V., et al., (2011) Journal of Pathology. 224(4):448-60).
Research Category
Apoptosis & Cancer
Research Sub Category
Developmental Signaling
This Anti-NOTCH 3/N3ECD Antibody, clone 1E4 is validated for use in Western Blotting and Immunocytochemistry and Electron Microscopy for the detection of NOTCH 3/N3ECD.

Qualité

Evaluated by Western Blotting in MCF-7 cell lysate.

Western Blotting Analysis: 1 µg/mL of this antibody detected NOTCH 3 & the ectodermal domain (N3ECD) in 10 µg of MCF-7 cell lysate.

Description de la cible

~260 and ~210 kDa observed
This antibody detects both the full length NOTCH 3 protein (~260 kDa) and the ectodermal domain N3ECD (~210 kDa).

Forme physique

Format: Purified
Protein G Purified
Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Stockage et stabilité

Stable for 1 year at 2-8°C from date of receipt.

Clause de non-responsabilité

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Code de la classe de stockage

12 - Non Combustible Liquids

Classe de danger pour l'eau (WGK)

WGK 1

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable


Certificats d'analyse (COA)

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Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.

Consulter la Bibliothèque de documents

Saara Tikka et al.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism, 32(12), 2171-2180 (2012-09-06)
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary vascular dementia caused by mutations in NOTCH3 gene. Pathology is manifested in small- and middle-sized arteries throughout the body, though primarily in cerebral white matter.
Notch3 signalling promotes tumour growth in colorectal cancer.
Serafin, Valentina, et al.
The Journal of Pathology, 224, 448-460 (2011)
A Joutel et al.
The Journal of clinical investigation, 105(5), 597-605 (2000-03-11)
Mutations in Notch3 cause CADASIL (cerebral autosomal dominant adult onset arteriopathy), which leads to stroke and dementia in humans. CADASIL arteriopathy is characterized by major alterations of vascular smooth muscle cells and the presence of specific granular osmiophilic deposits. Patients
Arturo I Machuca-Parra et al.
The Journal of experimental medicine, 214(8), 2271-2282 (2017-07-13)
Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a neurological syndrome characterized by small vessel disease (SVD), stroke, and vascular cognitive impairment and dementia caused by mutations in NOTCH3 No therapies are available for this condition. Loss of
Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis.
Joutel, A, et al.
Lancet, 358, 2049-2051 (2001)

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