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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. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, integral
Chromosomal Location of Human Ortholog: 19p13.12
Cellular Component: actin cytoskeleton; cytosol; endoplasmic reticulum membrane; extracellular region; Golgi membrane; nucleoplasm; plasma membrane; receptor complex
Molecular Function: cadherin binding; protein binding
Biological Process: Notch receptor processing; Notch signaling pathway; transcription initiation from RNA polymerase II promoter
Disease: Cerebral Arteriopathy, Autosomal Dominant, With Subcortical Infarcts And Leukoencephalopathy; Lateral Meningocele Syndrome; Myofibromatosis, Infantile, 2
Reference #:  Q9UM47 (UniProtKB)
Alt. Names/Synonyms: CADASIL; CASIL; Neurogenic locus notch homolog protein 3; NOTC3; Notch 3; Notch 3 extracellular truncation; Notch 3 intracellular domain; Notch homolog 3 (Drosophila); NOTCH3
Gene Symbols: NOTCH3
Molecular weight: 243,631 Da
Basal Isoelectric point: 5.18  Predict pI for various phosphorylation states
CST Pathways:  ESC Pluripotency and Differentiation  |  Notch Signaling
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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