C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity

C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity

Repeat expansion mutation in C9ORF72 is the most common cause of familial ALS. Here, the authors generate motor neurons from cells of patients with C9ORF72 mutations, and characterize changes in gene expression in these motor neurons compared to genetically corrected lines, which suggest that glutam...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Bhuvaneish T. Selvaraj, Matthew R. Livesey, Chen Zhao, Jenna M. Gregory, Owain T. James, Elaine M. Cleary, Amit K. Chouhan, Angus B. Gane, Emma M. Perkins, Owen Dando, Simon G. Lillico, Youn-Bok Lee, Agnes L. Nishimura, Urjana Poreci, Sai Thankamony, Meryll Pray, Navneet A. Vasistha, Dario Magnani, Shyamanga Borooah, Karen Burr, David Story, Alexander McCampbell, Christopher E. Shaw, Peter C. Kind, Timothy J. Aitman, C. Bruce A. Whitelaw, Ian Wilmut, Colin Smith, Gareth B. Miles, Giles E. Hardingham, David J. A. Wyllie, Siddharthan Chandran
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2018
Materias:
Science
Q
Acceso en línea:https://doaj.org/article/6a962a5b03a74471945829a86138ad2f
Etiquetas: Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
id oai:doaj.org-article:6a962a5b03a74471945829a86138ad2f
record_format dspace
spelling oai:doaj.org-article:6a962a5b03a74471945829a86138ad2f2021-12-02T17:31:43ZC9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity10.1038/s41467-017-02729-02041-1723https://doaj.org/article/6a962a5b03a74471945829a86138ad2f2018-01-01T00:00:00Zhttps://doi.org/10.1038/s41467-017-02729-0https://doaj.org/toc/2041-1723Repeat expansion mutation in C9ORF72 is the most common cause of familial ALS. Here, the authors generate motor neurons from cells of patients with C9ORF72 mutations, and characterize changes in gene expression in these motor neurons compared to genetically corrected lines, which suggest that glutamate receptor subunit GluA1 is dysregulated in this form of ALS.Bhuvaneish T. SelvarajMatthew R. LiveseyChen ZhaoJenna M. GregoryOwain T. JamesElaine M. ClearyAmit K. ChouhanAngus B. GaneEmma M. PerkinsOwen DandoSimon G. LillicoYoun-Bok LeeAgnes L. NishimuraUrjana PoreciSai ThankamonyMeryll PrayNavneet A. VasisthaDario MagnaniShyamanga BorooahKaren BurrDavid StoryAlexander McCampbellChristopher E. ShawPeter C. KindTimothy J. AitmanC. Bruce A. WhitelawIan WilmutColin SmithGareth B. MilesGiles E. HardinghamDavid J. A. WyllieSiddharthan ChandranNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-14 (2018)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Bhuvaneish T. Selvaraj
Matthew R. Livesey
Chen Zhao
Jenna M. Gregory
Owain T. James
Elaine M. Cleary
Amit K. Chouhan
Angus B. Gane
Emma M. Perkins
Owen Dando
Simon G. Lillico
Youn-Bok Lee
Agnes L. Nishimura
Urjana Poreci
Sai Thankamony
Meryll Pray
Navneet A. Vasistha
Dario Magnani
Shyamanga Borooah
Karen Burr
David Story
Alexander McCampbell
Christopher E. Shaw
Peter C. Kind
Timothy J. Aitman
C. Bruce A. Whitelaw
Ian Wilmut
Colin Smith
Gareth B. Miles
Giles E. Hardingham
David J. A. Wyllie
Siddharthan Chandran
C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
description Repeat expansion mutation in C9ORF72 is the most common cause of familial ALS. Here, the authors generate motor neurons from cells of patients with C9ORF72 mutations, and characterize changes in gene expression in these motor neurons compared to genetically corrected lines, which suggest that glutamate receptor subunit GluA1 is dysregulated in this form of ALS.
format article
author Bhuvaneish T. Selvaraj
Matthew R. Livesey
Chen Zhao
Jenna M. Gregory
Owain T. James
Elaine M. Cleary
Amit K. Chouhan
Angus B. Gane
Emma M. Perkins
Owen Dando
Simon G. Lillico
Youn-Bok Lee
Agnes L. Nishimura
Urjana Poreci
Sai Thankamony
Meryll Pray
Navneet A. Vasistha
Dario Magnani
Shyamanga Borooah
Karen Burr
David Story
Alexander McCampbell
Christopher E. Shaw
Peter C. Kind
Timothy J. Aitman
C. Bruce A. Whitelaw
Ian Wilmut
Colin Smith
Gareth B. Miles
Giles E. Hardingham
David J. A. Wyllie
Siddharthan Chandran
author_facet Bhuvaneish T. Selvaraj
Matthew R. Livesey
Chen Zhao
Jenna M. Gregory
Owain T. James
Elaine M. Cleary
Amit K. Chouhan
Angus B. Gane
Emma M. Perkins
Owen Dando
Simon G. Lillico
Youn-Bok Lee
Agnes L. Nishimura
Urjana Poreci
Sai Thankamony
Meryll Pray
Navneet A. Vasistha
Dario Magnani
Shyamanga Borooah
Karen Burr
David Story
Alexander McCampbell
Christopher E. Shaw
Peter C. Kind
Timothy J. Aitman
C. Bruce A. Whitelaw
Ian Wilmut
Colin Smith
Gareth B. Miles
Giles E. Hardingham
David J. A. Wyllie
Siddharthan Chandran
author_sort Bhuvaneish T. Selvaraj
title C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
title_short C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
title_full C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
title_fullStr C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
title_full_unstemmed C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
title_sort c9orf72 repeat expansion causes vulnerability of motor neurons to ca2+-permeable ampa receptor-mediated excitotoxicity
publisher Nature Portfolio
publishDate 2018
url https://doaj.org/article/6a962a5b03a74471945829a86138ad2f
work_keys_str_mv AT bhuvaneishtselvaraj c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT matthewrlivesey c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT chenzhao c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT jennamgregory c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT owaintjames c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT elainemcleary c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT amitkchouhan c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT angusbgane c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT emmamperkins c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT owendando c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT simonglillico c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT younboklee c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT agneslnishimura c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT urjanaporeci c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT saithankamony c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT meryllpray c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT navneetavasistha c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT dariomagnani c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT shyamangaborooah c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT karenburr c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT davidstory c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT alexandermccampbell c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT christophereshaw c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT peterckind c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT timothyjaitman c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT cbruceawhitelaw c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT ianwilmut c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT colinsmith c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT garethbmiles c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT gilesehardingham c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT davidjawyllie c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
AT siddharthanchandran c9orf72repeatexpansioncausesvulnerabilityofmotorneuronstoca2permeableampareceptormediatedexcitotoxicity
_version_ 1718380497041620992

Ejemplares similares