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Prevalence of hepatocellular carcinoma in alpha-1-antitrypsin deficiency

โœ Scribed by Theresa Propst; Albert Propst; Otto Dietze; Gert Judmaier; Herbert Braunsteiner; Wolfgang Vogel

Book ID
118566450
Publisher
Elsevier Science
Year
1994
Tongue
English
Weight
492 KB
Volume
21
Category
Article
ISSN
0168-8278

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## Liver injury in PiZZ cY,-antitrypsin &-AT) deficiency probably results from toxic effects of the abnormal a,- AT molecule accumulating within the ER of liver cells.

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Liver disease in alpha-1-antitrypsin (alpha1AT) deficiency is caused by a gain-of-toxic function mechanism engendered by the accumulation of a mutant glycoprotein in the endoplasmic reticulum (ER). The extraordinary degree of variation in phenotypical expression of this liver disease is believed to