Mechanism of action and therapeutic route for a muscular dystrophy caused by a genetic defect in lipid metabolism
oleh: 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
| Format: | Article |
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| Diterbitkan: | Nature Portfolio 2022-03-01 |
Deskripsi
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.