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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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Detalles Bibliográficos
Autores principales:	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.
Formato:	Texto
Lenguaje:	English
Publicado:	Elsevier 2009
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2723994/ https://www.ncbi.nlm.nih.gov/pubmed/19232354 http://dx.doi.org/10.1016/j.jmb.2009.01.069

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