Jagged 1 Proteins and Enzymes

Jagged 1 is a transmembrane glycoprotein in the Delta-Serrate-Lag-2 (DSL) family of ligands that activate Notch proteins. Jagged 1 is widely expressed in adult and fetal tissues. Jagged-Notch signaling specifies cell fate, regulates pattern formation, defines boundaries between different cell types, and modulates cell proliferation and differentiation. It plays a key role in vascular, hematopoietic, myogenic, and neurogenic development. Mutations in human Jagged 1 are the cause of Alagille syndrome, an autosomal-dominant disorder characterized by intrahepatic cholestasis and abnormalities of heart, eye, vertebrae, as well as a characteristic facial appearance.