Article
AMPK Complex Activation Promotes Sarcolemmal Repair in Dysferlinopathy.
Molecular therapy : the journal of the American Society of Gene Therapy - 8 Apr 2020
Ono Hiroya, Suzuki Naoki, Kanno Shin-Ichiro, Kawahara Genri, Izumi Rumiko, Takahashi Toshiaki, Kitajima Yasuo, Osana Shion, Nakamura Naoko, Akiyama Tetsuya, Ikeda Kensuke, Shijo Tomomi, Mitsuzawa Shio, Nagatomi Ryoichi, Araki Nobukazu, Yasui Akira, Warita Hitoshi, Hayashi Yukiko K, Miyake Katsuya, Aoki Masashi
Abstract excerpt
Mutations in dysferlin are responsible for a group of progressive, recessively inherited muscular dystrophies known as dysferlinopathies. Using recombinant proteins and affinity purification methods combined with liquid chromatography-tandem mass spectrometry (LC-MS/MS), we found that AMP-activated protein kinase (AMPK)γ1 was bound to a region of dysferlin located between the third and fourth C2 domains. Using...
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