Jag1 represses Notch activation in lateral supporting cells and inhibits an outer hair cell fate in the medial compartment of the developing cochlea
Sandra de Haan,
Agustin A. Corbat,
Christopher R. Cederroth
et al.
Abstract:Notch signaling regulates both inner and middle ear morphogenesis and establishes a strict pattern of sensory cells in the organ of Corti in the mammalian cochlea. Patients with Alagille syndrome have impaired Notch signaling (~94% with JAG1 mutations) resulting in sensorineural and conductive hearing loss. Here, we investigate the function of Jag1-mediated Notch activation in cochlear patterning and signaling using the Jag1 Nodder (Jag1Ndr/Ndr) mouse model of Alagille syndrome. Jag1Ndr/Ndr mice exhibited seve… Show more
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