Next Article in Journal
Association Analysis of 14 Candidate Gene Polymorphism with Depression and Stress among Gestational Diabetes Mellitus
Previous Article in Journal
The Molecular Genetics of Gordon Syndrome
Previous Article in Special Issue
Molecular Therapies for Choroideremia
Open AccessReview

Retinogenesis of the Human Fetal Retina: An Apical Polarity Perspective

1
Department of Ophthalmology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands
2
The Netherlands Institute for Neuroscience, Royal Netherlands Academy of Arts and Sciences, 1105 BA Amsterdam, The Netherlands
*
Author to whom correspondence should be addressed.
Genes 2019, 10(12), 987; https://doi.org/10.3390/genes10120987
Received: 21 August 2019 / Revised: 25 November 2019 / Accepted: 26 November 2019 / Published: 29 November 2019
(This article belongs to the Special Issue Molecular Therapies for Inherited Retinal Diseases)
The Crumbs complex has prominent roles in the control of apical cell polarity, in the coupling of cell density sensing to downstream cell signaling pathways, and in regulating junctional structures and cell adhesion. The Crumbs complex acts as a conductor orchestrating multiple downstream signaling pathways in epithelial and neuronal tissue development. These pathways lead to the regulation of cell size, cell fate, cell self-renewal, proliferation, differentiation, migration, mitosis, and apoptosis. In retinogenesis, these are all pivotal processes with important roles for the Crumbs complex to maintain proper spatiotemporal cell processes. Loss of Crumbs function in the retina results in loss of the stratified appearance resulting in retinal degeneration and loss of visual function. In this review, we begin by discussing the physiology of vision. We continue by outlining the processes of retinogenesis and how well this is recapitulated between the human fetal retina and human embryonic stem cell (ESC) or induced pluripotent stem cell (iPSC)-derived retinal organoids. Additionally, we discuss the functionality of in utero and preterm human fetal retina and the current level of functionality as detected in human stem cell-derived organoids. We discuss the roles of apical-basal cell polarity in retinogenesis with a focus on Leber congenital amaurosis which leads to blindness shortly after birth. Finally, we discuss Crumbs homolog (CRB)-based gene augmentation.
Keywords: apical polarity; crumbs complex; fetal retina; PAR complex; retinal organoids; retinogenesis; gene augmentation; adeno-associated virus (AAV); Leber congenital amaurosis apical polarity; crumbs complex; fetal retina; PAR complex; retinal organoids; retinogenesis; gene augmentation; adeno-associated virus (AAV); Leber congenital amaurosis
MDPI and ACS Style

Quinn, P.M.; Wijnholds, J. Retinogenesis of the Human Fetal Retina: An Apical Polarity Perspective. Genes 2019, 10, 987.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop