Fnip1 regulates skeletal muscle fiber type specification, fatigue resistance, and susceptibility to muscular dystrophy
Copyright policy )Fnip1 regulates skeletal muscle fiber type specification, fatigue resistance, and susceptibility to muscular dystrophy
Significance Folliculin interacting protein-1 (Fnip1) is an intracellular protein known to interact with folliculin (a protein mutated in Birt Hogg Dube’ Syndrome) and the master metabolic sensor AMP kinase. However, the roles of Fnip1 in mammalian development and function are unclear. In this study, we used mice deficient in Fnip1 to show that Fnip1 regulates skeletal muscle fiber type specification. Mice deficient in Fnip1 were significantly enriched for highly oxidative skeletal muscle that is more resistant to fatigue than wild-type muscle. Loss of Fnip1 also decreased muscle damage in a mouse model of Duchenne muscular dystrophy. These results reveal a previously unidentified function for Fnip1 and suggest that pharmacological inhibition of Fnip1 may reduce muscle damage in patients with muscular dystrophy.
- Fred Hutchinson Cancer Research Center United States
- University of Mary United States
- Fred Hutchinson Cancer Research Center South Africa
- University of Washington United States
Male, Mice, Knockout, Myosin Heavy Chains, Myoglobin, TOR Serine-Threonine Kinases, AMP-Activated Protein Kinases, Mechanistic Target of Rapamycin Complex 1, Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha, Mitochondria, Muscle, Muscular Dystrophy, Duchenne, Disease Models, Animal, Mice, Muscle Fibers, Slow-Twitch, Multiprotein Complexes, Muscle Fatigue, Muscle Fibers, Fast-Twitch, Mice, Inbred mdx, Animals, Carrier Proteins, Muscle Contraction
Male, Mice, Knockout, Myosin Heavy Chains, Myoglobin, TOR Serine-Threonine Kinases, AMP-Activated Protein Kinases, Mechanistic Target of Rapamycin Complex 1, Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha, Mitochondria, Muscle, Muscular Dystrophy, Duchenne, Disease Models, Animal, Mice, Muscle Fibers, Slow-Twitch, Multiprotein Complexes, Muscle Fatigue, Muscle Fibers, Fast-Twitch, Mice, Inbred mdx, Animals, Carrier Proteins, Muscle Contraction
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