The Z type variation of human α1-antitrypsin causes a protein folding defect

@article{Yu1995TheZT,
  title={The Z type variation of human α1-antitrypsin causes a protein folding defect},
  author={Myeong-Hee Yu and Kee Nyung Lee and Jeongho Kim},
  journal={Nature Structural Biology},
  year={1995},
  volume={2},
  pages={363-367}
}
Emphysema is often associated with the Z type mutation of α1,-antitrypsin, which causes aggregation of the molecule in the liver and consequent plasma deficiency. The aggregation appears to be due to loop-sheet polymerization, although why the mutant protein polymerizes in vivo is unclear. Here we show that, unlike wild type antitrypsin, which folds in minutes, the folding of Z type α1,-antitrypsin is extremely slow. Once folded, however, the native Z protein shows substantial stability towards… CONTINUE READING
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