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Notch signaling in the pigmented epithelium of the anterior eye segment promotes ciliary body development at the expense of iris formation
Author(s) -
Sarode Bhushan,
Nowell Craig S.,
Ihm JongEun,
Kostic Corinne,
Arsenijevic Yvan,
Moulin Alexandre P.,
Schorderet Daniel F.,
Beermann Friedrich,
Radtke Freddy
Publication year - 2014
Publication title -
pigment cell and melanoma research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.618
H-Index - 105
eISSN - 1755-148X
pISSN - 1755-1471
DOI - 10.1111/pcmr.12236
Subject(s) - ciliary body , iris (biosensor) , biology , aniridia , eye development , cilium , anterior eye segment , glaucoma , notch signaling pathway , optic cup (embryology) , phthisis bulbi , anatomy , microbiology and biotechnology , signal transduction , neuroscience , cornea , genetics , transcription factor , computer security , computer science , gene , biometrics
Summary The ciliary body and iris are pigmented epithelial structures in the anterior eye segment that function to maintain correct intra‐ocular pressure and regulate exposure of the internal eye structures to light, respectively. The cellular and molecular factors that mediate the development of the ciliary body and iris from the ocular pigmented epithelium remain to be fully elucidated. Here, we have investigated the role of Notch signaling during the development of the anterior pigmented epithelium by using genetic loss‐ and gain‐of‐function approaches. Loss of canonical Notch signaling results in normal iris development but absence of the ciliary body. This causes progressive hypotony and over time leads to phthisis bulbi, a condition characterized by shrinkage of the eye and loss of structure/function. Conversely, Notch gain‐of‐function results in aniridia and profound ciliary body hyperplasia, which causes ocular hypertension and glaucoma‐like disease. Collectively, these data indicate that Notch signaling promotes ciliary body development at the expense of iris formation and reveals novel animal models of human ocular pathologies.