Notch 3 (NOTCH3) (C-Term) antibody

Antigen: Notch 3 (NOTCH3)

Synonyms: CASIL, CADASIL

Binding Site: C-Term

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Conjugate: Un-conjugated

Application: Immunohistochemistry (IHC)

Catalog no.: ABIN966682

Additional Information

Alternative name: NOTCH3

Immunogen: Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to C-terminal residues of human NOTCH3 (Neurogenic locus notch homolog protein 3)

Description: NOTCH3 (Neurogenic locus notch homolog protein 3) 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 RBP-J kappa and activates genes of the enhancer of split locus. NOTCH3 affects the implementation of differentiation, proliferation and apoptotic programs. NOTCH3 is a heterodimer of a C-terminal fragment N(TM) and a N-terminal fragment N(EC) which are probably linked by disulfide bonds. NOTCH3 interacts with MAML1, MAML2 and MAML3 which act as transcriptional coactivators for NOTCH3. NOTCH3 is ubiquitously expressed in fetal and adult tissues. It is 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. Defects in NOTCH3 are the cause of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). CADASIL causes a type of stroke and dementia of which key features include recurrent subcortical ischemic events and vascular dementia. The disorder affects relatively young adults of both sexes.

Application Details

Restrictions: For Research Use only

Publications

Product: Joutel, Corpechot, Ducros et al.: "Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia." in: Nature, Vol. 383, Issue 6602, pp. 707-10, 1996 (PubMed).

Gray, Mann, Mitsiadis et al.: "Human ligands of the Notch receptor." in: The American journal of pathology, Vol. 154, Issue 3, pp. 785-94, 1999 (PubMed).

Wu, Sun, Kobayashi et al.: "Identification of a family of mastermind-like transcriptional coactivators for mammalian notch receptors." in: Molecular and cellular biology, Vol. 22, Issue 21, pp. 7688-700, 2002 (PubMed).