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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Main Authors: 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.
Format: Artigo
Jezik:Inglês
Izdano: Elsevier 2009
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Article
Online dostop: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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