Tamoxifen therapy in a murine model of myotubular myopathy

Myotubular myopathy is a severe muscle disease for which no effective treatment exists. Here, the authors show that tamoxifen ameliorates pathology and extends survival in a mouse model of the disease, and that the effect is mediated via estrogen receptor signaling and involves modulation of DNM2 ex...

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Autores principales: Nika Maani, Nesrin Sabha, Kamran Rezai, Arun Ramani, Linda Groom, Nadine Eltayeb, Faranak Mavandadnejad, Andrea Pang, Giulia Russo, Michael Brudno, Volker Haucke, Robert T. Dirksen, James J. Dowling
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Publicado: Nature Portfolio 2018
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Acceso en línea:https://doaj.org/article/7fbfa0258973415ea77898bbaf016988
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spelling oai:doaj.org-article:7fbfa0258973415ea77898bbaf0169882021-12-02T17:31:27ZTamoxifen therapy in a murine model of myotubular myopathy10.1038/s41467-018-07057-52041-1723https://doaj.org/article/7fbfa0258973415ea77898bbaf0169882018-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-07057-5https://doaj.org/toc/2041-1723Myotubular myopathy is a severe muscle disease for which no effective treatment exists. Here, the authors show that tamoxifen ameliorates pathology and extends survival in a mouse model of the disease, and that the effect is mediated via estrogen receptor signaling and involves modulation of DNM2 expression.Nika MaaniNesrin SabhaKamran RezaiArun RamaniLinda GroomNadine EltayebFaranak MavandadnejadAndrea PangGiulia RussoMichael BrudnoVolker HauckeRobert T. DirksenJames J. DowlingNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-16 (2018)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Nika Maani
Nesrin Sabha
Kamran Rezai
Arun Ramani
Linda Groom
Nadine Eltayeb
Faranak Mavandadnejad
Andrea Pang
Giulia Russo
Michael Brudno
Volker Haucke
Robert T. Dirksen
James J. Dowling
Tamoxifen therapy in a murine model of myotubular myopathy
description Myotubular myopathy is a severe muscle disease for which no effective treatment exists. Here, the authors show that tamoxifen ameliorates pathology and extends survival in a mouse model of the disease, and that the effect is mediated via estrogen receptor signaling and involves modulation of DNM2 expression.
format article
author Nika Maani
Nesrin Sabha
Kamran Rezai
Arun Ramani
Linda Groom
Nadine Eltayeb
Faranak Mavandadnejad
Andrea Pang
Giulia Russo
Michael Brudno
Volker Haucke
Robert T. Dirksen
James J. Dowling
author_facet Nika Maani
Nesrin Sabha
Kamran Rezai
Arun Ramani
Linda Groom
Nadine Eltayeb
Faranak Mavandadnejad
Andrea Pang
Giulia Russo
Michael Brudno
Volker Haucke
Robert T. Dirksen
James J. Dowling
author_sort Nika Maani
title Tamoxifen therapy in a murine model of myotubular myopathy
title_short Tamoxifen therapy in a murine model of myotubular myopathy
title_full Tamoxifen therapy in a murine model of myotubular myopathy
title_fullStr Tamoxifen therapy in a murine model of myotubular myopathy
title_full_unstemmed Tamoxifen therapy in a murine model of myotubular myopathy
title_sort tamoxifen therapy in a murine model of myotubular myopathy
publisher Nature Portfolio
publishDate 2018
url https://doaj.org/article/7fbfa0258973415ea77898bbaf016988
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