In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome

Abstract The photoreceptor-specific nuclear receptor Nr2e3 is not expressed in Nr2e3 rd7/rd7 mice, a mouse model of the recessively inherited retinal degeneration enhanced S-cone sensitivity syndrome (ESCS). We characterized in detail C57BL/6J Nr2e3 rd7/rd7 mice in vivo by fundus photography, optica...

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Autores principales:	Giulia Venturini, Despina Kokona, Beatrice L. Steiner, Emanuele G. Bulla, Joel Jovanovic, Martin S. Zinkernagel, Pascal Escher
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/998c07516489425aa2d964b03d8eb876
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spelling	oai:doaj.org-article:998c07516489425aa2d964b03d8eb8762021-12-02T17:27:19ZIn vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome10.1038/s41598-021-98271-72045-2322https://doaj.org/article/998c07516489425aa2d964b03d8eb8762021-09-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-98271-7https://doaj.org/toc/2045-2322Abstract The photoreceptor-specific nuclear receptor Nr2e3 is not expressed in Nr2e3 rd7/rd7 mice, a mouse model of the recessively inherited retinal degeneration enhanced S-cone sensitivity syndrome (ESCS). We characterized in detail C57BL/6J Nr2e3 rd7/rd7 mice in vivo by fundus photography, optical coherence tomography and fluorescein angiography and, post mortem, by histology and immunohistochemistry. White retinal spots and so-called ‘rosettes’ first appear at postnatal day (P) 12 in the dorsal retina and reach maximal expansion at P21. The highest density in ‘rosettes’ is observed within a region located between 100 and 350 µM from the optic nerve head. ‘Rosettes’ disappear between 9 to 12 months. Non-apoptotic cell death markers are detected during the slow photoreceptor degeneration, at a rate of an approximately 3% reduction of outer nuclear layer thickness per month, as observed from 7 to 31 months of age. In vivo analysis of Nr2e3 rd7/rd7 Cx3cr1 gfp/+ retinas identified microglial cells within ‘rosettes’ from P21 on. Subretinal macrophages were observed in vivo and by confocal microscopy earliest in 12-months-old Nr2e3 rd7/rd7 retinas. At P21, S-opsin expression and the number of S-opsin expressing dorsal cones was increased. The dorso-ventral M-cone gradient was present in Nr2e3 rd7/rd7 retinas, but M-opsin expression and M-opsin expressing cones were decreased. Retinal vasculature was normal.Giulia VenturiniDespina KokonaBeatrice L. SteinerEmanuele G. BullaJoel JovanovicMartin S. ZinkernagelPascal EscherNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-14 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Giulia Venturini Despina Kokona Beatrice L. Steiner Emanuele G. Bulla Joel Jovanovic Martin S. Zinkernagel Pascal Escher In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome
description	Abstract The photoreceptor-specific nuclear receptor Nr2e3 is not expressed in Nr2e3 rd7/rd7 mice, a mouse model of the recessively inherited retinal degeneration enhanced S-cone sensitivity syndrome (ESCS). We characterized in detail C57BL/6J Nr2e3 rd7/rd7 mice in vivo by fundus photography, optical coherence tomography and fluorescein angiography and, post mortem, by histology and immunohistochemistry. White retinal spots and so-called ‘rosettes’ first appear at postnatal day (P) 12 in the dorsal retina and reach maximal expansion at P21. The highest density in ‘rosettes’ is observed within a region located between 100 and 350 µM from the optic nerve head. ‘Rosettes’ disappear between 9 to 12 months. Non-apoptotic cell death markers are detected during the slow photoreceptor degeneration, at a rate of an approximately 3% reduction of outer nuclear layer thickness per month, as observed from 7 to 31 months of age. In vivo analysis of Nr2e3 rd7/rd7 Cx3cr1 gfp/+ retinas identified microglial cells within ‘rosettes’ from P21 on. Subretinal macrophages were observed in vivo and by confocal microscopy earliest in 12-months-old Nr2e3 rd7/rd7 retinas. At P21, S-opsin expression and the number of S-opsin expressing dorsal cones was increased. The dorso-ventral M-cone gradient was present in Nr2e3 rd7/rd7 retinas, but M-opsin expression and M-opsin expressing cones were decreased. Retinal vasculature was normal.
format	article
author	Giulia Venturini Despina Kokona Beatrice L. Steiner Emanuele G. Bulla Joel Jovanovic Martin S. Zinkernagel Pascal Escher
author_facet	Giulia Venturini Despina Kokona Beatrice L. Steiner Emanuele G. Bulla Joel Jovanovic Martin S. Zinkernagel Pascal Escher
author_sort	Giulia Venturini
title	In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome
title_short	In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome
title_full	In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome
title_fullStr	In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome
title_full_unstemmed	In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome
title_sort	in vivo analysis of onset and progression of retinal degeneration in the nr2e3 rd7/rd7 mouse model of enhanced s-cone sensitivity syndrome
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/998c07516489425aa2d964b03d8eb876
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In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 rd7/rd7 mouse model of enhanced S-cone sensitivity syndrome

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