1-antitrypsin deficiency pathology introduced

α1-antitrypsin deficiency pathology introduced

α1-antitrypsin (α1-AT) is of a low molecular weight glycoprotein synthesized by the liver is the major component of the serum alpha globulin, inhibit trypsin and other proteases effect, it is known as trypsin. The synthetic gene of α1-AT Pi in the long arm of chromosome 14. α1-antitrypsin deficiency is an autosomal dominant genetic disease, children with more than after the birth of the performance of the hepatitis syndrome.


α1-antitrypsin a serious lack of homozygous seen Pizz, moderate lack seen Piss homozygous PiSZ to-and PiME heterozygous. The ATD of α-plasma. α1-AT content decreased synthesis obstacles may be related to liver cells, release from the liver to reduce the amount of human plasma, and the synthesis of α-AT molecule structural abnormalities. Normal alpha-AT homozygous PiMM genotype, called MM-type α1-AT. Abnormal pathological α1-AT and normal α1-AT different is the arrangement of the polypeptide chain, small molecular weight and low solubility, so that the liver can not be the excretion of the circulating blood, and massive accumulation of rough endoplasmic reticulum in liver cells . Because the normal α1-AT can inhibit trypsin and a variety of proteolytic enzymes, the protective effect from the organization, so the blood of normal α1-AT rarely, liver and other organs may be damaged. Abnormal α1-AT deposition in the endoplasmic reticulum in liver cells, can form the inclusion of PAS positive granules, damage to liver cells Erzhi liver disease, and finally can be caused by α1-antitrypsin deficiency cirrhosis.

 

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