Crystallographic and Cellular Characterisation of Two Mechanisms Stabilising the Native Fold of α(1)-Antitrypsin: Implications for Disease and Drug Design

The common Z mutant (Glu342Lys) of α(1)-antitrypsin results in the formation of polymers that are retained within hepatocytes. This causes liver disease whilst the plasma deficiency of an important proteinase inhibitor predisposes to emphysema. The Thr114Phe and Gly117Phe mutations border a surface...

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Detaylı Bibliyografya
Asıl Yazarlar: Gooptu, Bibek, Miranda, Elena, Nobeli, Irene, Mallya, Meera, Purkiss, Andrew, Leigh Brown, Sarah C., Summers, Charlotte, Phillips, Russell L., Lomas, David A., Barrett, Tracey E.
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: Elsevier 2009
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Article
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC2723994/
https://ncbi.nlm.nih.gov/pubmed/19232354
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jmb.2009.01.069
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