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Using Stem Cells to Model Diseases of the Outer Retina.

Yvon, C; Ramsden, CM; Lane, A; Powner, MB; da Cruz, L; Coffey, PJ; Carr, AJ; (2015) Using Stem Cells to Model Diseases of the Outer Retina. Computational and Structural Biotechnology Journal , 13 pp. 382-389. 10.1016/j.csbj.2015.05.001. Green open access

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Abstract

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).

Type: Article
Title: Using Stem Cells to Model Diseases of the Outer Retina.
Location: Sweden
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.csbj.2015.05.001
Publisher version: http://dx.doi.org/10.1016/j.csbj.2015.05.001
Language: English
Additional information: © 2015 Yvon et al. Published by Elsevier B.V. on behalf of the Research Network of Computational and Structural Biotechnology. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
Keywords: Age related macular degeneration, Disease models, Induced pluripotent stem cells, Inherited retinopathy, Leber congenital amaurosis, Retinitis pigmentosa
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/1472676
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