Adaptational role of mitochondrial changes in congenital central core myopathy: new proofs

The objective of the investigation was to study the ultrastructural morphology of muscle tissue mitochondria versus other clinical and laboratory indicators in 21 patients with congenital central core myopathy. The findings demonstrate that the sizes of mitochondria are correlated with the levels of lactate and pyruvate; however, the magnitude and direction of this correlation differ in the subsarco-lemmal and intermyofibrillar mitochondrial fractions. It is concluded that the baseline level of lactic acid in the blood of the patients depends on the number of mitochondria whereas the maturity of organelles largely provides a tissue response to elevated lactic acid levels. Our data are evidence in favor of an assumption that rounded mitochondria are less mature and effective in performing then-main functions. The increase in the number of ellipsoidal organelles in the subsarcolemmal region of muscle fibers is ascertained to be an important compensatory mechanism in nonmitochondrial muscle diseases. Thus, the investigation has established the role of changes in the number and sizes of mitochondria in tissue adaptation to the conditions of functional insufficiency. The found m

children, myopathy, central core myopathy, mitochondrion, adaptation, subsarcolemmal fraction, intermyqflbrillar fraction

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