Gipertrofica cardiomyopathy (SCOP), one of the major diseases attack involving his kidney. Refers to the under-researched primary heart disease of unknown etiology characterized by the walls of overdetermination turned left without expansion cavity sistolicescoy increased and the violation diastolicescoy functions. In general population SCOP is 0.2%, or one out of every 500 people. SCOP is one of the most common hereditary diseases of the circulatory system. Along with a long stable condition gipertrofica cardiomyopathy can be compounded sudden death, acute and chronic heart failure, jizneopasnami disorders heart rhythm. Aetiology and pathogenesis of the modern view SCOP is predominantly genetically caused disease muscle disease, which is characterized by a set of specific morfofunktionalnah changes and steadily evolving over a high risk of severe jizneugrojath arrhythmias and sudden death (COM). SCOP is a massive (more than 1.5 cm) overdetermination attack left, and / or, in rare cases, the right heart, increasingly asymmetric nature through excessive mejjeludockova wall (MJP), the frequent development obstruction (systolic pressure gradient) output tract turned left (LV), in the absence of known causes (HBP, and the evils of specific heart disease). The main method of diagnosis remains ehocardiograficescoe study. Characteristically gipercontraktilnoe the attack at normal or reduced LV cavity until its obliteration in sistolu. There is a general recognition of the concept of mostly hereditary nature SCOP. The literature widespread term "family гипертрофическая cardiomyopathy." To date, found that more than half of all cases are Neogene, and the principle is the inheritance autosomno-dominantna . The rest of the so-called sporadic form; In this case, the patient no relatives SCOP ill or suffering attacks. It is believed that most, if not all cases of sporadic SCOP also have a genetic cause, or caused by random mutations. SCOP is a genetically heterogeneous disease, which had led to more than 200 mutations described at least nine genes encoding proteins миофибриллярного apparatus. So far, 10 known protein components of a heart sarkomera performing contraktilnuu, structural or regulatory functions, defects which were in the Majnoon. And in each gene mutations many can cause disease (poligennoe multiallelnoe disease). Defects protein components in SCOP 50-85% of all mutations About 15-20% of all mutations Heavy chain бета-миозина (35-50%) Эссенциальная and regulatory light chain миозина Тропонин T (15-20%) Альфа-тропомиозин Миозинсвязывающий protein C (15-20%) Альфа-актин God I тропонин Heavy chain альфа-миозина Титин Mutations in the gene lead to the synthesis of mutant proteins included in the apparatus miofibrillarnyi cells, where they have a negative impact on the reduction and / or myocardial relaxation. One of the main manifestations patofiziologicakih SCOP-disorder oxidative metabolism, which contributes to the development of the energy deficit in cardiomiotitah. Mutations of genes sarkomernykh proteins can enhance increased miofibrillarna Atf-aza, causing excess waste energy while reducing. Mutations of genes energy metabolism-induced АМФ-активируемой протеинкиназы (CIDA). CIDA-marker deficit cellular energy, and one of the major thesis of energy "in the heart, which controls the oxidation of fatty acids and glucose entry into cells.