hereditary disease, which is based on genetically from the exchange of copper with its excess accumulation mainly in the liver and the nervous system. Aetiology and pathogenesis. The reason is autosomno-retessivno Inheritance of copper metabolism; gene is located in a long part of chromosome 13. Prevalence in the different regions of the world, on average, graduated with an geterozigotny carrying about 1%. The gene is expressed in the liver, kidney, platzente. Produced gene is a kationtransportirut s R-tip Atfaznogo protein. The genetic defect is a different degree of the violation of the intracellular transport of copper. This leads to a decline in copper excretion in jelchew and accumulation in gepatocitah. With food a day is 2-5 mg copper. She absorbed in the gut are directed to the liver, which is associated with liver sinteziruemam zerouloplazminom circulates in the serum, selectively caught organs and excreted from jelchew. As standard copper excretion jelchew with 2 mg / day, with a Villson-konovalova only 0.2-0.4 mg, which leads to an increased accumulation of copper in the body. The copper in ceruloplasmin happening in the office Goldji involving gene gepatotserebralna malnutrition. A small portion of the copper in the blood in ionic form, as a labile complex with albuminom and out with the urine. The disease Villson-konovalova increased copper absorption in the gut, reduced excretion of copper with jelchew. Lower copper excretion linked to a gene defect gepatotserebralna malnutrition defining transport copper in Goldji apparatus and the subsequent allocation lizosomami in elixirs. Breached the incorporation of copper in ceruloplasmin. Because of the lack of use of a copper deposit it in the liver, brain, kidney, cornea. Deposited in the liver secondary copper ingibiruet ceruloplasmine synthesis. Ceruloplasmine level in the serum is diagnostic, but not pathogenetic importance. The 5% of the patients normal ceruloplasmine. In the liver biopsy of the patients had excessive copper, as increased copper content in the blood and tissues, it is with urine. Copper, a prooksidantom has a toxic effect on the organism. The accumulation leads to increased production of free radicals formed. In a survey of patients Villson-konovalova disease and animals with the experimental shipping copper in plasma determined by the reduction of vitamin E, the increase in gasoline products peroxide oxidation of lipids; In the liver decreased levels of reduced metabolism and vitamin E. Pap liver are targets of oxidants. Violation of the respiratory chain and the slowdown Zitohrom-s-oksidasy increases production of free radicals leaks electrons from the respiratory chain. Free copper accumulate in the tissues, Sh-group blocking enzymes involved in the okislitiono-vosstan Ideology reactions. This leads to energy starving to which most sensitive central nervous system. At the beginning of the clinical features absent (stage I), copper accumulates in zitozole liver cells. Copper-related Sh-groupami zitozolnah proteins, making gepatocitami secretion of proteins and triglycerides. There steatos hepatocytes and the emergence of cells Mallory. In the second stage copper redeployed from zitozolya lizosoma in hepatocytes. Part comes in the blood. Due to the low specific activity lizosom biliarnaya copper excretion lowered. Copper is pereokislenie lipids and damage lysosomal membranes before tackling harmful acid gidrolaz in zitoplazmu. There necrosis of hepatocytes, the development of chronic hepatitis and hemolytic anemia. In stage III increased copper accumulation in the liver leads to fibrozu and cirrhosis. Increased accumulation of copper in the brain, cornea, distal renal divisions Channel has deployed picture disease.
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