In situ study of myofibrils, mitochondria and bound creatine kinases in experimental cardiomyopathies.

Human cardiomyopathy has been extensively studied in the last decade, and knowledge of the functional and structural alterations of the heart has grown. However, understanding of the pathogenesis has come mostly from experimental studies. A number of work have been designed to elucidate if alterations of the contractile apparatus of cardiac cells contribute to the impairment of heart mechanics in cardiomyopathies. As well, an important question is to be solved: whether energy supply of the contraction-relaxation cycle is sufficient in the myopathic heart. Use of cardiac fibers skinned by different techniques allows to evaluate functional ability of myofibrils, mitochondria and bound creatine kinase which plays an important role in cardiomyocyte energy metabolism. The data presented in this chapter show that experimental cardiomyopathies of various types have some common features. These are an increase in calcium sensitivity of myofibrils and a depression of functional activity of mitochondrial creatine kinase. Possible mechanisms and physiological significance of these changes are discussed.