A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1‐antitrypsin deficiency

@article{Miranda2010ANM,
  title={A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1‐antitrypsin deficiency},
  author={E. Miranda and J. P{\'e}rez and U. Ekeowa and N. Had{\vz}i{\'c} and N. Kalsheker and B. Gooptu and B. Portmann and D. Belorgey and M. Hill and Susan Chambers and J. Teckman and G. Alexander and S. Marciniak and D. Lomas},
  journal={Hepatology},
  year={2010},
  volume={52}
}
  • E. Miranda, J. Pérez, +11 authors D. Lomas
  • Published 2010
  • Biology, Medicine
  • Hepatology
  • Alpha1‐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 pathological polymers formed by α1‐antitrypsin. This antibody was used to characterize the Z variant… CONTINUE READING
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