Nature Communications (Mar 2022)
Mechanism of action and therapeutic route for a muscular dystrophy caused by a genetic defect in lipid metabolism
- Mahtab Tavasoli,
- Sarah Lahire,
- Stanislav Sokolenko,
- Robyn Novorolsky,
- Sarah Anne Reid,
- Abir Lefsay,
- Meredith O. C. Otley,
- Kitipong Uaesoontrachoon,
- Joyce Rowsell,
- Sadish Srinivassane,
- Molly Praest,
- Alexandra MacKinnon,
- Melissa Stella Mammoliti,
- Ashley Alyssa Maloney,
- Marina Moraca,
- J. Pedro Fernandez-Murray,
- Meagan McKenna,
- Christopher J. Sinal,
- Kanneboyina Nagaraju,
- George S. Robertson,
- Eric P. Hoffman,
- Christopher R. McMaster
Affiliations
- Mahtab Tavasoli
- Department of Pharmacology, Dalhousie University
- Sarah Lahire
- University of Reims Champagne-Ardenne
- Stanislav Sokolenko
- Department of Process Engineering & Applied Science, Dalhousie University
- Robyn Novorolsky
- Department of Pharmacology, Dalhousie University
- Sarah Anne Reid
- Department of Pharmacology, Dalhousie University
- Abir Lefsay
- Mass Spectrometry Core Facility, Dalhousie University
- Meredith O. C. Otley
- Department of Pharmacology, Dalhousie University
- Kitipong Uaesoontrachoon
- Agada Biosciences Inc.
- Joyce Rowsell
- Agada Biosciences Inc.
- Sadish Srinivassane
- Agada Biosciences Inc.
- Molly Praest
- Agada Biosciences Inc.
- Alexandra MacKinnon
- Agada Biosciences Inc.
- Melissa Stella Mammoliti
- Agada Biosciences Inc.
- Ashley Alyssa Maloney
- Agada Biosciences Inc.
- Marina Moraca
- Agada Biosciences Inc.
- J. Pedro Fernandez-Murray
- Department of Pharmacology, Dalhousie University
- Meagan McKenna
- Agada Biosciences Inc.
- Christopher J. Sinal
- Department of Pharmacology, Dalhousie University
- Kanneboyina Nagaraju
- Agada Biosciences Inc.
- George S. Robertson
- Department of Pharmacology, Dalhousie University
- Eric P. Hoffman
- Agada Biosciences Inc.
- Christopher R. McMaster
- Department of Pharmacology, Dalhousie University
- DOI
- https://doi.org/10.1038/s41467-022-29270-z
- Journal volume & issue
-
Vol. 13,
no. 1
pp. 1 – 20
Abstract
Mutations in the CHKB gene cause muscular dystrophy. Here, the authors show that in mouse models of the disease changes in lipid metabolism are associated with decreased PPAR signaling, and show PPAR agonists can rescue expression of injury markers in myocytes in vitro.
