Anti-NOTCH 3/N3ECD Antibody, clone 1E4

产品说明

一般描述

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.

特异性

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

免疫原

His-tagged recombinant protein corresponding to Human NOTCH 3.

应用

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.
Research Category
Apoptosis & Cancer
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 Sub Category
Developmental Signaling

质量

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.

目标描述

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

外形

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

储存及稳定性

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

免责声明

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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