A combined in silico and in vitro study on mouse Serpina1a antitrypsin-deficiency mutants
Author:
Publisher
Springer Science and Business Media LLC
Subject
Multidisciplinary
Link
http://www.nature.com/articles/s41598-019-44043-3.pdf
Reference45 articles.
1. Camelier, A. A. et al. Alpha-1 antitrypsin deficiency: diagnosis and treatment. J Bras Pneumol 34, 514–527 (2008).
2. Ordonez, A. et al. Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency. Hepatology 57, 2049–2060, https://doi.org/10.1002/hep.26173 (2013).
3. Tafaleng, E. N. et al. Induced pluripotent stem cells model personalized variations in liver disease resulting from alpha1-antitrypsin deficiency. Hepatology 62, 147–157, https://doi.org/10.1002/hep.27753 (2015).
4. Segeritz, C. P. et al. hiPSC hepatocyte model demonstrates the role of unfolded protein response and inflammatory networks in alpha1-antitrypsin deficiency. J Hepatol, https://doi.org/10.1016/j.jhep.2018.05.028 (2018).
5. Carlson, J. A. et al. Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice. J Clin Invest 83, 1183–1190, https://doi.org/10.1172/JCI113999 (1989).
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