Article
Cyclophilin D, a target for counteracting skeletal muscle dysfunction in mitochondrial myopathy.
Human molecular genetics - 1 Dec 2015
Gineste Charlotte, Hernandez Andres, Ivarsson Niklas, Cheng Arthur J, Naess Karin, Wibom Rolf, Lesko Nicole, Bruhn Helene, Wedell Anna, Freyer Christoph, Zhang Shi-Jin, Carlström Mattias, Lanner Johanna T, Andersson Daniel C, Bruton Joseph D, Wredenberg Anna, Westerblad Håkan
Abstract excerpt
Muscle weakness and exercise intolerance are hallmark symptoms in mitochondrial disorders. Little is known about the mechanisms leading to impaired skeletal muscle function and ultimately muscle weakness in these patients. In a mouse model of lethal mitochondrial myopathy, the muscle-specific Tfam knock-out (KO) mouse, we previously demonstrated an excessive mitochondrial Ca(2+) uptake in isolated muscle fibers...
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