Article
Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes.
The Journal of clinical investigation - 1 May 2011
Ding Jianqiang, Yannam Govardhana R, Roy-Chowdhury Namita, Hidvegi Tunda, Basma Hesham, Rennard Stephen I, Wong Ronald J, Avsar Yesim, Guha Chandan, Perlmutter David H, Fox Ira J, Roy-Chowdhury Jayanta
Abstract excerpt
α1-Antitrypsin deficiency is an inherited condition that causes liver disease and emphysema. The normal function of this protein, which is synthesized by the liver, is to inhibit neutrophil elastase, a protease that degrades connective tissue of the lung. In the classical form of the disease, inefficient secretion of a mutant α1-antitrypsin protein (AAT-Z) results in its accumulation within hepatocytes and...
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