C. elegans is an extremely powerful model organism to study gene function and biology. The nematodes’ genetic tractability and high degree of genetic similarity with humans make it ideal to study the biologic role of serpins. The endogenous C. elegans serpins are most similar to the human clade B or intracellular serpin family and have a predominantly cytoplasmic distribution. Since the nematode genes often have functional orthologues in humans, the biological role of mammalian serpins can be extrapolated from C. elegans. Additionally, mutant-mammalian serpin genes resulting in serpinopathies can be introduced into C. elegans to determine the biological pathways that play a role in disease progression, as well as develop potential therapeutics to ameliorate the disease.

Original languageEnglish
Title of host publicationThe Serpin Family
Subtitle of host publicationProteins with Multiple Functions in Health and Disease
PublisherSpringer International Publishing
Number of pages16
ISBN (Electronic)9783319227115
ISBN (Print)9783319227108
StatePublished - Jan 1 2015


  • ATD
  • Caenorhabditis elegans
  • Cathepsin
  • Clade B (or intracellular) serpins
  • Intracellular serpin
  • Model organism
  • Necrotic cell death (or NCD) serpinopathies
  • SRP-1
  • SRP-2
  • SRP-3
  • SRP-6
  • SRP-7
  • α1-antitrypsin


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