Proteolytic cleavage of the long pentraxin PTX3 in the airways of cystic fibrosis patients

Yveline Hamon, Sébastien Jaillon, Christine Person, Jean Louis Giniès, Erwan Garo, Barbara Bottazzi, Sarah Ghamrawi, Thierry Urban, Jean François Subra, Jean Philippe Bouchara, Alberto Mantovani, Pascale Jeannin, Yves Delneste

Research output: Contribution to journalArticlepeer-review


The prototypic long pentraxin PTX3, a soluble pattern recognition receptor, plays an important role in innate defense against selected pathogens by favoring their elimination and the initiation of protective responses. PTX3 has notably beneficial effects in mice infected with Aspergillus fumigatus and Pseudomonas aeruginosa. Cystic fibrosis (CF), a severe inherited autosomal recessive disease, is characterized by recurrent lung infections, especially by these two pathogens. We thus hypothesized that the status of PTX3 may be altered in CF patients. Level and integrity of PTX3 were analyzed in the sputum samples from 51 CF patients and 7 patients with chronic obstructive pulmonary disease (COPD). The levels of PTX3 were increased in serums from CF patients, but low in their respiratory secretions. PTX3 concentrations in sputum samples were dramatically lower in CF patients than in COPD patients. The low concentration of PTX3 resulted from a proteolysis cleavage by elastase and A. fumigatus proteases. Interestingly, the N-ter domain of PTX3, involved in protection against A. fumigatus, is preferentially degraded by these proteases. These results indicate that the selective proteolysis of PTX3 in the CF lung may explain, in part, the recurrent lung infections by PTX3-sensitive pathogens in CF patients.

Original languageEnglish
Pages (from-to)611-622
Number of pages12
JournalInnate Immunity
Issue number6
Publication statusPublished - Dec 2013


  • Aspergillus fumigatus
  • cystic fibrosis
  • elastase
  • pentraxin 3

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology
  • Immunology
  • Microbiology
  • Infectious Diseases


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