ABSTRACT
The muscle, as in any living cell, many chemical reactions such as synthesis of biological macromolecules, active transport of molecules across cell membranes, production of force and movement, require a continual input of energy. The muscle function involves the integrity of metabolic pathways that ensure the synthesis of adenosine triphosphate [ATP], especially during the transition from resting state to that of a maximum contraction, it also regenerated from either exogenous oxidative fuels [fatty acids, carbohydrates], or from the body reserves of [glycogen]. The metabolic dysfunctions, it means the enzymic deficiencies lead to a metabolic myopathy. The main causes of ATP deficiency and its consequences on the muscle cells function, relate to deficiencies in beta-oxidation of fatty acids, those of glycolysis and the mitochondrial respiratory chain