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Conformational Properties of the Disease-Causing Z Variant of α1-Antitrypsin Revealed by Theory and Experiment
The human serine protease inhibitor (serpin) α-1 antitrypsin (α1-AT) protects tissues from proteases of inflammatory cells. The most common disease-causing mutation in α1-AT is the Z-mutation (E342K) that results in an increased propensity of α1-AT to polymerize in the ER of hepatocytes, leading to...
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| Main Authors: | , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
The Biophysical Society
2012
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3379022/ https://ncbi.nlm.nih.gov/pubmed/22735536 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bpj.2012.05.023 |
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