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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Bibliographic Details
Main Authors: Kass, Itamar, Knaupp, Anja S., Bottomley, Stephen P., Buckle, Ashley M.
Format: Artigo
Language:Inglês
Published: The Biophysical Society 2012
Subjects:
Protein
Online Access: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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