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Jagged 1 (JAG1) is a cell surface protein that functions in an embryologically important signaling pathway, known as the Notch signaling pathway, which is a highly conserved cell signaling system.1 JAG1 plays an essential role in tissue formation during embryonic development of primitive organisms.2 JAG1 is a strong candidate gene for Alagille syndrome (AGS)3 which is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region.4