Article
A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.
Hepatology (Baltimore, Md.) - 1 Sept 2010
Miranda Elena, Pérez Juan, Ekeowa Ugo I, Hadzic Nedim, Kalsheker Noor, Gooptu Bibek, Portmann Bernard, Belorgey Didier, Hill Marian, Chambers Susan, Teckman Jeff, Alexander Graeme J, Marciniak Stefan J, Lomas David A
Abstract excerpt
UNLABELLED: Alpha(1)-antitrypsin is the most abundant circulating protease inhibitor. The severe Z deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. This causes neonatal hepatitis, cirrhosis, and hepatocellular carcinoma. We have developed a conformation-specific monoclonal antibody (2C1) that recognizes the...
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