产品货号 :  mlR51148

英文名称 :  NOTCH3

中文名称 :  跨膜受体蛋白Notch-3单克隆抗体

别    名 :  CADASIL; CASIL; NOTC3_HUMAN; Notch 3; Notch 3 intracellular domain; Notch homolog 3; Notch3.  

抗体来源 :  Mouse

克隆类型 :  Monoclonal

克 隆 号 :  6G2

交叉反应 :   Human,

产品应用 :  WB=1:100-500  

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  255kDa

细胞定位 :  细胞核 细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human NOTCH3:2291-2321

亚    型 :  IgG

纯化方法 :  affinity purified by Protein G

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed :  PubMed

产品介绍 :   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]

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.

Subunit:

Heterodimer of a C-terminal fragment N(TM) and a N-terminal fragment N(EC) which are probably linked by disulfide bonds. Interacts with MAML1, MAML2 and MAML3 which act as transcriptional coactivators for NOTCH3. Interacts with PSMA1. Interacts with HIF1AN.

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 modifications:

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.

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.

Myofibromatosis, 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.

Similarity:

Belongs to the NOTCH family.

Contains 5 ANK repeats.

Contains 34 EGF-like domains.

Contains 3 LNR (Lin/Notch) repeats.

SWISS:

Q9UM47

Gene ID:

4854

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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