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...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Nature Portfolio
2018-11-01
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| Series: | Nature Communications |
| Online Access: | https://doi.org/10.1038/s41467-018-07057-5 |
| _version_ | 1818978191280177152 |
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| 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 |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-20T16:39:43Z |
| format | Article |
| id | doaj.art-7fbfa0258973415ea77898bbaf016988 |
| institution | Directory Open Access Journal |
| issn | 2041-1723 |
| language | English |
| last_indexed | 2024-12-20T16:39:43Z |
| publishDate | 2018-11-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Nature Communications |
| spelling | doaj.art-7fbfa0258973415ea77898bbaf0169882022-12-21T19:33:04ZengNature PortfolioNature Communications2041-17232018-11-019111610.1038/s41467-018-07057-5Tamoxifen therapy in a murine model of myotubular myopathyNika Maani0Nesrin Sabha1Kamran Rezai2Arun Ramani3Linda Groom4Nadine Eltayeb5Faranak Mavandadnejad6Andrea Pang7Giulia Russo8Michael Brudno9Volker Haucke10Robert T. Dirksen11James J. Dowling12Program for Genetics and Genome Biology, Hospital for Sick ChildrenProgram for Genetics and Genome Biology, Hospital for Sick ChildrenProgram for Genetics and Genome Biology, Hospital for Sick ChildrenProgram for Genetics and Genome Biology, Hospital for Sick ChildrenDepartment of Pharmacology and Physiology, University of Rochester Medical Center School of Medicine and DentistryProgram for Genetics and Genome Biology, Hospital for Sick ChildrenProgram for Genetics and Genome Biology, Hospital for Sick ChildrenProgram for Genetics and Genome Biology, Hospital for Sick ChildrenDepartment of Molecular Pharmacology and Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie (FMP)Program for Genetics and Genome Biology, Hospital for Sick ChildrenDepartment of Molecular Pharmacology and Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie (FMP)Department of Pharmacology and Physiology, University of Rochester Medical Center School of Medicine and DentistryProgram for Genetics and Genome Biology, Hospital for Sick ChildrenMyotubular 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.https://doi.org/10.1038/s41467-018-07057-5 |
| spellingShingle | 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 Nature Communications |
| title | 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_short | Tamoxifen therapy in a murine model of myotubular myopathy |
| title_sort | tamoxifen therapy in a murine model of myotubular myopathy |
| url | https://doi.org/10.1038/s41467-018-07057-5 |
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