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1
Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology.
Thorax. 2004 Jun;59(6):529-35. doi: 10.1136/thx.2003.006528.
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Molecular pathogenesis of alpha-1-antitrypsin deficiency.
Rev Mal Respir. 2014 Dec;31(10):992-1002. doi: 10.1016/j.rmr.2014.03.015. Epub 2014 Nov 20.
5
A transgenic zebrafish model of hepatocyte function in human Z α1-antitrypsin deficiency.
Biol Chem. 2019 Nov 26;400(12):1603-1616. doi: 10.1515/hsz-2018-0391.
7
The molecular species responsible for α -antitrypsin deficiency are suppressed by a small molecule chaperone.
FEBS J. 2021 Apr;288(7):2222-2237. doi: 10.1111/febs.15597. Epub 2020 Nov 11.
9
Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency.
COPD. 2013 Mar;10 Suppl 1:17-25. doi: 10.3109/15412555.2013.764401.
10
Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema.
Am J Respir Cell Mol Biol. 1998 May;18(5):670-4. doi: 10.1165/ajrcmb.18.5.3065.

引用本文的文献

3
Prevalence of Cardiovascular Disease and Rate of Major Adverse Cardiovascular Events in Severe Alpha-1 Antitrypsin Deficiency COPD.
Int J Chron Obstruct Pulmon Dis. 2024 Jan 17;19:149-159. doi: 10.2147/COPD.S419846. eCollection 2024.
4
Utility of the Serum Protein Electrophoresis in the Opportunistic Screening for the Deficiency of Alpha-1 Antitrypsin.
Diagnostics (Basel). 2023 Aug 28;13(17):2778. doi: 10.3390/diagnostics13172778.
6
Recombinant Alpha-1 Antitrypsin as Dry Powder for Pulmonary Administration: A Formulative Proof of Concept.
Pharmaceutics. 2022 Dec 8;14(12):2754. doi: 10.3390/pharmaceutics14122754.
9
Gene therapy for alpha 1-antitrypsin deficiency with an oxidant-resistant human alpha 1-antitrypsin.
JCI Insight. 2020 Aug 6;5(15):135951. doi: 10.1172/jci.insight.135951.
10
Molecular diagnosis of alpha1-antitrypsin deficiency: A new method based on Luminex technology.
J Clin Lab Anal. 2020 Jul;34(7):e23279. doi: 10.1002/jcla.23279. Epub 2020 Mar 17.

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EXPERIMENTAL EMPHYSEMA: ITS PRODUCTION WITH PAPAIN IN NORMAL AND SILICOTIC RATS.
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[alpha1-Antitrypsin from human serum].
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Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease.
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Nucleation of alpha 1-antichymotrypsin polymerization.
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Drosophila necrotic mutations mirror disease-associated variants of human serpins.
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Serpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerol.
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Serpinopathies and the conformational dementias.
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Interactions causing the kinetic trap in serpin protein folding.
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