Adaptation of striated muscles to Wolframin deficiency in mice: Alterations in cellular bioenergetics.

Kersti Tepp,Marju Puurand,Natalja Timohhina,Jekaterina Aid-Vanakova,Indrek Reile,Igor Shevchuk,Vladimir Chekulayev,Margus Eimre,Nadežda Peet,Lumme Kadaja,Kalju Paju,Tuuli Käämbre

Biochimica et Biophysica Acta (BBA) - General Subjects（2020）

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摘要

•Increased mitochondrial proton leak in glycolytic muscles in wolframin deficiency.•Glutamate oxidation declines in muscles in wolframin deficiency.•Wolframin deficiency affects coupling between mitochondria and glycolysis.•Wolframin deficient cardiac muscle has altered energy transfer.

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