Using Stem Cells to Model Diseases of the Outer Retina

Camille Yvon; Conor M. Ramsden; Amelia Lane; Michael B. Powner; Lyndon da Cruz; Peter J. Coffey; Amanda-Jayne F. Carr

doi:10.1016/j.csbj.2015.05.001

Computational and Structural Biotechnology Journal (Jan 2015)

Using Stem Cells to Model Diseases of the Outer Retina

Camille Yvon,
Conor M. Ramsden,
Amelia Lane,
Michael B. Powner,
Lyndon da Cruz,
Peter J. Coffey,
Amanda-Jayne F. Carr

Affiliations

Camille Yvon: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK
Conor M. Ramsden: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK
Amelia Lane: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK
Michael B. Powner: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK
Lyndon da Cruz: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK
Peter J. Coffey: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK
Amanda-Jayne F. Carr: The London Project to Cure Blindness, Division of ORBIT, Institute of Ophthalmology, University College London, 11-43 Bath Street, London EC1V 9EL, UK

DOI: https://doi.org/10.1016/j.csbj.2015.05.001
Journal volume & issue: Vol. 13, no. C
pp. 382 – 389

Abstract

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Retinal degeneration arises from the loss of photoreceptors or retinal pigment epithelium (RPE). It is one of the leading causes of irreversible blindness worldwide with limited effective treatment options. Generation of induced pluripotent stem cell (IPSC)-derived retinal cells and tissues from individuals with retinal degeneration is a rapidly evolving technology that holds a great potential for its use in disease modelling. IPSCs provide an ideal platform to investigate normal and pathological retinogenesis, but also deliver a valuable source of retinal cell types for drug screening and cell therapy. In this review, we will provide some examples of the ways in which IPSCs have been used to model diseases of the outer retina including retinitis pigmentosa (RP), Usher syndrome (USH), Leber congenital amaurosis (LCA), gyrate atrophy (GA), juvenile neuronal ceroid lipofuscinosis (NCL), Best vitelliform macular dystrophy (BVMD) and age related macular degeneration (AMD).

Published in Computational and Structural Biotechnology Journal

ISSN: 2001-0370 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Technology: Chemical technology: Biotechnology
Website: https://www.journals.elsevier.com/computational-and-structural-biotechnology-journal

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