Resumen

HAMANA, Leticia  y  SUAREZ, Claudia. Patología mitocondrial en las enfermedades del miocardio. Gac Méd Caracas [online]. 2002, vol.110, n.4, pp.478-493. ISSN 0367-4762.

Mutations in the mitochondrial genome may occur in any organ or system. The cardiac function same as those functions of other organs, such as the skeleton muscle and the brain depends on the energy generated by the mitochondrias mainly by the oxidation of the fat acids and carbohydrates through the mechanism of oxidative phosphorylation. All proteins coded by the mitochondrial genome are subunits of the mechanism of oxidative phosphorylation complex, in such a way that mutations of DNAmit aid alterations of this crucial process which are declared in the heart with myocardial diseases (cardiomyopathies), disorders of conduction and occasionally, letal arrhythmias. All diseases of the cardiac muscle circulate with structural and functional changes of mitochondrias under variable degree, the mitochondrias themselves may be the origin of a serie of specific alterations of the cardiac muscle (mitochondrials cardiomyhopathies) and other organs. The importance of this lies in the fact that adequate therapeutics may arise upon knowing its role in the normal/abnormal cardiac function. In consequence, it is important to advise about the "not uncommon existence" of such disorders.

Palabras clave : Mitochondrias; Mitochondrials cardiomyhopathies; Mitochondrial deoxyribonucleic acid.