Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models

Mutations in the Retinitis Pigmentosa GTPase Regulator (RPGR) cause retinal dystrophy, but how this arises at a molecular level is unclear. Here, the authors show in induced pluripotent stem cells and mouse knockouts that RPGR mediates actin dynamics in photoreceptors via the actin-severing protein,...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Roly Megaw, Hashem Abu-Arafeh, Melissa Jungnickel, Carla Mellough, Christine Gurniak, Walter Witke, Wei Zhang, Hemant Khanna, Pleasantine Mill, Baljean Dhillon, Alan F. Wright, Majlinda Lako, Charles ffrench-Constant
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2017
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/8c1cade21905425da1a4b4301e31ca1c
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:8c1cade21905425da1a4b4301e31ca1c
record_format	dspace
spelling	oai:doaj.org-article:8c1cade21905425da1a4b4301e31ca1c2021-12-02T14:16:14ZGelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models10.1038/s41467-017-00111-82041-1723https://doaj.org/article/8c1cade21905425da1a4b4301e31ca1c2017-08-01T00:00:00Zhttps://doi.org/10.1038/s41467-017-00111-8https://doaj.org/toc/2041-1723Mutations in the Retinitis Pigmentosa GTPase Regulator (RPGR) cause retinal dystrophy, but how this arises at a molecular level is unclear. Here, the authors show in induced pluripotent stem cells and mouse knockouts that RPGR mediates actin dynamics in photoreceptors via the actin-severing protein, gelsolin.Roly MegawHashem Abu-ArafehMelissa JungnickelCarla MelloughChristine GurniakWalter WitkeWei ZhangHemant KhannaPleasantine MillBaljean DhillonAlan F. WrightMajlinda LakoCharles ffrench-ConstantNature PortfolioarticleScienceQENNature Communications, Vol 8, Iss 1, Pp 1-10 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Roly Megaw Hashem Abu-Arafeh Melissa Jungnickel Carla Mellough Christine Gurniak Walter Witke Wei Zhang Hemant Khanna Pleasantine Mill Baljean Dhillon Alan F. Wright Majlinda Lako Charles ffrench-Constant Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
description	Mutations in the Retinitis Pigmentosa GTPase Regulator (RPGR) cause retinal dystrophy, but how this arises at a molecular level is unclear. Here, the authors show in induced pluripotent stem cells and mouse knockouts that RPGR mediates actin dynamics in photoreceptors via the actin-severing protein, gelsolin.
format	article
author	Roly Megaw Hashem Abu-Arafeh Melissa Jungnickel Carla Mellough Christine Gurniak Walter Witke Wei Zhang Hemant Khanna Pleasantine Mill Baljean Dhillon Alan F. Wright Majlinda Lako Charles ffrench-Constant
author_facet	Roly Megaw Hashem Abu-Arafeh Melissa Jungnickel Carla Mellough Christine Gurniak Walter Witke Wei Zhang Hemant Khanna Pleasantine Mill Baljean Dhillon Alan F. Wright Majlinda Lako Charles ffrench-Constant
author_sort	Roly Megaw
title	Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
title_short	Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
title_full	Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
title_fullStr	Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
title_full_unstemmed	Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
title_sort	gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models
publisher	Nature Portfolio
publishDate	2017
url	https://doaj.org/article/8c1cade21905425da1a4b4301e31ca1c
work_keys_str_mv	AT rolymegaw gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT hashemabuarafeh gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT melissajungnickel gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT carlamellough gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT christinegurniak gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT walterwitke gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT weizhang gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT hemantkhanna gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT pleasantinemill gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT baljeandhillon gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT alanfwright gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT majlindalako gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels AT charlesffrenchconstant gelsolindysfunctioncausesphotoreceptorlossininducedpluripotentcellandanimalretinitispigmentosamodels
_version_	1718391680151846912

Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models

Ejemplares similares