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Molecular basis of α(1)-antitrypsin deficiency revealed by the structure of a domain-swapped trimer
α(1)-Antitrypsin (α1AT) deficiency is a disease with multiple manifestations, including cirrhosis and emphysema, caused by the accumulation of stable polymers of mutant protein in the endoplasmic reticulum of hepatocytes. However, the molecular basis of misfolding and polymerization remain unknown....
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| 主要な著者: | , , , , |
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| フォーマット: | Artigo |
| 言語: | Inglês |
| 出版事項: |
Nature Publishing Group
2011
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| 主題: | |
| オンライン･アクセス: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3185345/ https://ncbi.nlm.nih.gov/pubmed/21909074 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/embor.2011.171 |
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