Polymers and inflammation: disease mechanisms of the serpinopathies

Members of the serpin (serine proteinase inhibitor) superfamily play a central role in the control of inflammatory, coagulation, and fibrinolytic cascades. Point mutations that cause abnormal conformational transitions in these proteins can trigger disease. Recent work has defined three pathways by which these conformers cause tissue damage. Here, we describe how these three mechanisms can be integrated into a new model of the pathogenesis of emphysema caused by mutations in the serpin α1-antitrypsin.

• Publication year

  2008

• Venue

  Journal of Experimental Medicine

• Publication date

  2008-07-07

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1084/jem.20072080PMID 18591408PMCID 2442629
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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