The role of adiponectin has been particularly deepened in diabetic muscles while the study of adiponectin in hereditary myop- athies has been marginally investigated. Here, we report the study about adiponectin effects in Col6a1−/− (collagen VI–null) mice. Col6a1−/− mice show myophatic phenotype closer to that of patients with Bethlem myopathy, thus representing an excellent animal model for the study of this hereditary disease. Our findings demonstrate that Col6a1−/− mice have decreased plasma adiponectin content and diseased myoblasts have an impaired autocrine secretion of the hormone. Moreover, Col6a1−/− myo- blasts show decreased glucose uptake and mitochondria with depolarized membrane potential and impaired functionality, as supported by decreased oxygen consumption. Exogenous addition of globular adiponectin modifies the features of Col6a1−/− myoblasts, becoming closer to that of the healthy myoblasts. Indeed, globular adiponectin enhances glucose uptake in Col6a1−/− myoblasts, modifies mitochondrial membrane potential, and restores oxygen consumption, turning closer to those of wild-type myoblasts. Finally, increase of plasma adiponectin level in Col6a1−/− mice is induced by fasting, a condition that has been previously shown to lead to the amelioration of the dystrophic phenotype. Collectively, our results demonstrate that exogenous replenishment of adiponectin reverses metabolic abnormalities observed in Col6a1−/− myoblasts.

Role of adiponectin in the metabolism of skeletal muscles in collagen VI–related myopathies

Martina Chrisam;Matilde Cescon;Silvia Castagnaro;Paola Braghetta;
2019

Abstract

The role of adiponectin has been particularly deepened in diabetic muscles while the study of adiponectin in hereditary myop- athies has been marginally investigated. Here, we report the study about adiponectin effects in Col6a1−/− (collagen VI–null) mice. Col6a1−/− mice show myophatic phenotype closer to that of patients with Bethlem myopathy, thus representing an excellent animal model for the study of this hereditary disease. Our findings demonstrate that Col6a1−/− mice have decreased plasma adiponectin content and diseased myoblasts have an impaired autocrine secretion of the hormone. Moreover, Col6a1−/− myo- blasts show decreased glucose uptake and mitochondria with depolarized membrane potential and impaired functionality, as supported by decreased oxygen consumption. Exogenous addition of globular adiponectin modifies the features of Col6a1−/− myoblasts, becoming closer to that of the healthy myoblasts. Indeed, globular adiponectin enhances glucose uptake in Col6a1−/− myoblasts, modifies mitochondrial membrane potential, and restores oxygen consumption, turning closer to those of wild-type myoblasts. Finally, increase of plasma adiponectin level in Col6a1−/− mice is induced by fasting, a condition that has been previously shown to lead to the amelioration of the dystrophic phenotype. Collectively, our results demonstrate that exogenous replenishment of adiponectin reverses metabolic abnormalities observed in Col6a1−/− myoblasts.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11577/3294867
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