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Retarded protein folding of deficient human α(1)-antitrypsin D256V and L41P variants
α(1)-Antitrypsin is the most abundant protease inhibitor in plasma and is the archetype of the serine protease inhibitor superfamily. Genetic variants of human α(1)-antitrypsin are associated with early-onset emphysema and liver cirrhosis. However, the detailed molecular mechanism for the pathogenic...
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| Main Authors: | , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado: |
Cold Spring Harbor Laboratory Press
2004
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| Acceso en liña: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2286720/ https://ncbi.nlm.nih.gov/pubmed/14767073 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1110/ps.03356604 |
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