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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.
- Source :
-
Pigment cell & melanoma research [Pigment Cell Melanoma Res] 2014 Jul; Vol. 27 (4), pp. 580-9. Date of Electronic Publication: 2014 Apr 07. - Publication Year :
- 2014
-
Abstract
- 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.<br /> (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Subjects :
- Animals
Ciliary Body cytology
Eye Proteins genetics
Humans
Iris cytology
Mice
Mice, Transgenic
Pigment Epithelium of Eye cytology
Receptors, Notch genetics
Ciliary Body embryology
Eye Proteins metabolism
Iris embryology
Pigment Epithelium of Eye embryology
Receptors, Notch metabolism
Signal Transduction physiology
Subjects
Details
- Language :
- English
- ISSN :
- 1755-148X
- Volume :
- 27
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Pigment cell & melanoma research
- Publication Type :
- Academic Journal
- Accession number :
- 24628886
- Full Text :
- https://doi.org/10.1111/pcmr.12236