Nature Communications (Nov 2018)

Tamoxifen therapy in a murine model of myotubular myopathy

  • 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

DOI
https://doi.org/10.1038/s41467-018-07057-5
Journal volume & issue
Vol. 9, no. 1
pp. 1 – 16

Abstract

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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.