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MABC594

Sigma-Aldrich

Anti-NOTCH 3/N3ECD Antibody, clone 1E4

clone 1E4, 1 mg/mL, from mouse

Synonym(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

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

biological source

mouse

Quality Level

100

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

1E4, monoclonal

species reactivity

human

concentration

1 mg/mL

technique(s)

electron microscopy: suitable
immunocytochemistry: suitable
western blot: suitable

isotype

IgG1κ

NCBI accession no.

NP_000426

UniProt accession no.

Q9UM47

shipped in

wet ice

target post-translational modification

unmodified

Gene Information

human ... NOTCH3(4854)

General description

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.

Specificity

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

Immunogen

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.

Quality

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.

Target description

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

Physical form

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.

Storage and Stability

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

Disclaimer

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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Storage Class Code

12 - Non Combustible Liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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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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