Article
Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.
The Journal of clinical investigation - 1 Apr 1999
Mahadeva R, Chang W S, Dafforn T R, Oakley D J, Foreman R C, Calvin J, Wight D G, Lomas D A
Abstract excerpt
The association between Z alpha1-antitrypsin deficiency and juvenile cirrhosis is well-recognized, and there is now convincing evidence that the hepatic inclusions are the result of entangled polymers of mutant Z alpha1-antitrypsin. Four percent of the northern European Caucasian population are heterozygotes for the Z variant, but even more common is S alpha1-antitrypsin, which is found in up to 28% of southern...
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