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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Hlavní autoři: 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.
Médium: Artigo
Jazyk:Inglês
Vydáno: Elsevier 2009
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On-line přístup: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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