's general knowledge of cause of disease of the plump type myocardium disease

's general knowledge of cause of disease of the plump type myocardium disease
Plump type myocardium disease (hypertrophic cardiomyopathy, HCM) Diminish as characteristic with myocardium the intersection of asymmetry and plump, the intersection of ventricle and, blood plentiful to obstruct with left ventricle, relaxation and a complying with myocardium disease which drops to the basic morbid state. According to the epidemiology materials, the persons who there is family's history account for 50%, men and women proportion 2: 1,Have illness coming on for 38 persons of age and 15 years old on average. This disease is often the reason why the youth dies suddenly.
Cause of disease
50% of the plump type myocardium disease patients are caused by albumen gene mutation of myocardium sarcomere, there is family's history, are considered to be dominant hereditary disease of the autosome. The pathogenic mechanism of about 50% of the patients is still indeterminate.
(1) Family plump type myocardium disease
The gene has already verified that there are 7 genes, more than 70 kinds of mutation that correlate with plump type myocardium disease: 8_ myoglobulin heavy chain (MHC) The gene, myocardium troponin a T (cardiac troponin T, cTnT) The gene, one's tropomyosin (tropomyosin) The gene, the conjugated proteln a C (myosin binding protien-C) of myoglobulin ; The expression products of some pathogenic genes of 7q3 location have not been fixed yet. Click in three pieces of genetic location of genetic affirmation of the code sarcomere albumen: Myoglobulin light chain vMLC-1, vMLC-2, troponin I (cTnI) . Myocardium disease know four chromosome that pathogenic genetic location order show in 3-10-2 Fig. already. The sudden change of mitochondrial DNA relates to family plump type myocardium disease too, such as mtRNA (glycine) 9997th nucleotide mutation of gene can cause the plump type myocardium disease.
(2) Plump promotion factor of the cardiac muscle
Have already found the plump type myocardium disease patient catecholamine strengthens the storage amount with phosphoric acid adenosine of ring to reduce actively. Add noradrenaline people's myocardium cells to train in the liquid, find myc cancer gene is duplicated and increased by 5- 10 times horizontally in myocardium cells, and promote the cardiac muscle to be plump, this reaction can be blocked pharmaceutical by a receptor and checked, can also be strengthened by protein kinase C activator, briefing on noradrenaline makes myc cancer gene expression increase through the fact that Q receptor activates the lipoprotein of phosphoinositide protein kinase C system, the original cancer gene may be starting to move one of the factors of plump type myocardium disease. Discover, 33% heart interventricular septum and calcium of the atrium skin resist the pharmaceutical receptor to increase short of moneyly in the plump type myocardium disease patient, afterbirth the intersection of calcium and regulation mechanism might participate in getting plump myocardium the intersection of disease and course of having illness coming on while being unusual in the thick liquid. Noradrenaline, heart sodium are plain to increase with brain sodium peptide density notably in the plump type myocardium disease patient blood plasma, brain sodium peptide density can reflect the pressure steps are bad and the left ventricle relaxation function is not complete in the ventricle, heart sodium plain to reflect left ventricle relaxation function the whole only.
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