Article
Mice expressing L345P mutant desmin exhibit morphological and functional changes of skeletal and cardiac mitochondria.
Journal of muscle research and cell motility - 1 Jan 2008
Kostareva Anna, Sjöberg Gunnar, Bruton Joseph, Zhang Shi-Jin, Balogh Johanna, Gudkova Alexandra, Hedberg Birgitta, Edström Lars, Westerblad Håkan, Sejersen Thomas
Abstract excerpt
Desmin mutations underlie inherited myopathies/cardiomyopathies with varying severity and involvement of the skeletal and cardiac muscles. We developed a transgenic mouse model expressing low level of the L345P desmin mutation (DESMUT mice) in order to uncover changes in skeletal and cardiac muscles caused by this mutation. The most striking ultrastructural changes in muscle from DESMUT mice were mitochondrial...
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