抗体

NOTCH3多克隆抗体

MBS126597

0.05毫升

230美元

多克隆

兔

未共轭

人类, 小鼠, 大鼠

免疫印迹, 免疫组化, 免疫细胞化学

抗体

NOTCH3多克隆抗体

NOTCH3

CASIL;遗传性多发梗死痴呆病

Neurogenic locus notch homolog protein 3; Neurogenic locus notch homolog protein 3; neurogenic locus notch homolog protein 3; Notch homolog 3; notch 3

NOTCH3

NOTCH3; NOTCH3; IMF2; CASIL; CADASIL; Notch 3

NOTC3_HUMAN

多克隆

IgG

兔

人类, 小鼠, 大鼠

2321

亲和纯化

免疫印迹(免疫印迹), 免疫组化(免疫组化), 免疫荧光(IF)

Species: Human. Route: Synthetic Peptide

Immunogen: A synthetic peptide of human NOTCH3

多克隆

Notch proteins (Notch1-4) are a family of transmembrane receptors that play important roles in development and the determination of cell fate (1). Mature Notch receptors are processed and assembled as heterodimeric proteins, with each dimer comprised of a large extracellular ligand-binding domain, a single-pass transmembrane domain, and a smaller cytoplasmic subunit (Notch intracellular domain, NICD) (2). Binding of Notch receptors to ligands of the Delta-Serrate-Lag2 (DSL) family triggers heterodimer dissociation, exposing the receptors to proteolytic cleavages; these result in release of the NICD, which translocates to the nucleus and activates transcription of downstream target genes (3-4). Notch3 is a member of the Notch family and is processed similar to Notch1 (5). It is expressed primarily in arterial smooth muscle cells (SMC). Mutations altering the number of cysteine residues in the Notch3 extracellular region are associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a hereditary angiopathy leading to strokes and dementia in adults (6-8). Recent studies indicate that Notch3 is overexpressed in many types of cancer (9-11).

322510053

Q9UM47.2

Q9UM47

2321

Delta-Notch Signaling Pathway (198879); Dorso-ventral Axis Formation Pathway (114227); Dorso-ventral Axis Formation Pathway (472); Gene Expression Pathway (105937); Generic Transcription Pathway (105938); MicroRNAs In Cancer Pathway (852705); MicroRNAs In Cancer Pathway (852928); Neural Crest Differentiation Pathway (672460); Notch Signaling Pathway (198891); Notch Signaling Pathway (1084758)

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]

NOTCH3: 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. 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. Ubiquitously expressed in fetal and adult tissues. Belongs to the NOTCH family. Protein type: Membrane protein, integral. Chromosomal Location of Human Ortholog: 19p13.2-p13.1. Cellular Component: Golgi membrane; nucleoplasm; endoplasmic reticulum membrane; cytoplasm; plasma membrane; extracellular region; integral to membrane; cytosol; receptor complex; actin cytoskeleton. Molecular Function: protein binding; enzyme binding; calcium ion binding. Biological Process: transcription initiation from RNA polymerase II promoter; negative regulation of neuron differentiation; Notch signaling pathway; regulation of transcription, DNA-dependent; positive regulation of smooth muscle cell proliferation; forebrain development; neuron fate commitment; Notch receptor processing; gene expression. Disease: Cerebral Arteriopathy, Autosomal Dominant, With Subcortical Infarcts And Leukoencephalopathy; Lateral Meningocele Syndrome; Myofibromatosis, Infantile, 2

0.05毫升

230美元

0.1毫升

305

0.2毫升

460