Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells

Angelman syndrome (AS) is characterized by developmental delay and intellectual disability, but the underlying pathophysiology is not well understood. Here the authors use induced pluripotent stem cell-derived neurons from AS patients and find impaired maturation of resting membrane potential and ac...

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Autores principales:	James J. Fink, Tiwanna M. Robinson, Noelle D. Germain, Carissa L. Sirois, Kaitlyn A. Bolduc, Amanda J. Ward, Frank Rigo, Stormy J. Chamberlain, Eric S. Levine
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2017
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/1375ce3c32de4a919e6c93e690fd3493
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spelling	oai:doaj.org-article:1375ce3c32de4a919e6c93e690fd34932021-12-02T14:42:55ZDisrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells10.1038/ncomms150382041-1723https://doaj.org/article/1375ce3c32de4a919e6c93e690fd34932017-04-01T00:00:00Zhttps://doi.org/10.1038/ncomms15038https://doaj.org/toc/2041-1723Angelman syndrome (AS) is characterized by developmental delay and intellectual disability, but the underlying pathophysiology is not well understood. Here the authors use induced pluripotent stem cell-derived neurons from AS patients and find impaired maturation of resting membrane potential and action potential firing, and defects in synaptic activity associated with the disease.James J. FinkTiwanna M. RobinsonNoelle D. GermainCarissa L. SiroisKaitlyn A. BolducAmanda J. WardFrank RigoStormy J. ChamberlainEric S. LevineNature PortfolioarticleScienceQENNature Communications, Vol 8, Iss 1, Pp 1-14 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q James J. Fink Tiwanna M. Robinson Noelle D. Germain Carissa L. Sirois Kaitlyn A. Bolduc Amanda J. Ward Frank Rigo Stormy J. Chamberlain Eric S. Levine Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells
description	Angelman syndrome (AS) is characterized by developmental delay and intellectual disability, but the underlying pathophysiology is not well understood. Here the authors use induced pluripotent stem cell-derived neurons from AS patients and find impaired maturation of resting membrane potential and action potential firing, and defects in synaptic activity associated with the disease.
format	article
author	James J. Fink Tiwanna M. Robinson Noelle D. Germain Carissa L. Sirois Kaitlyn A. Bolduc Amanda J. Ward Frank Rigo Stormy J. Chamberlain Eric S. Levine
author_facet	James J. Fink Tiwanna M. Robinson Noelle D. Germain Carissa L. Sirois Kaitlyn A. Bolduc Amanda J. Ward Frank Rigo Stormy J. Chamberlain Eric S. Levine
author_sort	James J. Fink
title	Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells
title_short	Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells
title_full	Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells
title_fullStr	Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells
title_full_unstemmed	Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells
title_sort	disrupted neuronal maturation in angelman syndrome-derived induced pluripotent stem cells
publisher	Nature Portfolio
publishDate	2017
url	https://doaj.org/article/1375ce3c32de4a919e6c93e690fd3493
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Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells

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