Defining the mechanism of polymerization in the serpinopathies

The serpinopathies result from the ordered polymerization of mutants of members of the serine proteinase inhibitor (serpin) superfamily. These polymers are retained within the cell of synthesis where they cause a toxic gain of function. The serpinopathies are exemplified by inclusions that form with...

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Bibliographic Details
Main Authors: Ekeowa, Ugo I., Freeke, Joanna, Miranda, Elena, Gooptu, Bibek, Bush, Matthew F., Pérez, Juan, Teckman, Jeff, Robinson, Carol V., Lomas, David A.
Format: Artigo
Language:Inglês
Published: National Academy of Sciences 2010
Subjects:
Biological Sciences
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC2951428/
https://ncbi.nlm.nih.gov/pubmed/20855577
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1004785107
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