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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

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Published on: April 11, 2018

Modifications of plasma fibronectin in cystic fibrosis patients

L Stanislawski1, M Sorin

  • 1Institut National de la Santé et de la Recherche Médicale (U.294), CHU 7 Xavier Bichat, Université Paris 7, France.

Infection and Immunity
|March 1, 1991
PubMed

Insights

Cystic fibrosis (CF) patients show reduced binding of fibronectin (FN) to bacteria like Staphylococcus aureus. This altered fibronectin may contribute to recurrent lung infections in CF, impacting respiratory health.

Area of Science:

  • Pulmonary Medicine
  • Microbiology
  • Biochemistry

Background:

  • Recurrent pulmonary infections by Staphylococcus aureus and Pseudomonas aeruginosa are a significant challenge in cystic fibrosis (CF).
  • These infections lead to progressive respiratory deterioration in CF patients.
  • Fibronectin (FN), a protein involved in cellular adhesion, possesses binding sites for bacteria such as S. aureus.

Purpose of the Study:

  • To investigate the bacterial binding characteristics of fibronectin (FN) from cystic fibrosis (CF) patients.
  • To compare CF-derived FN with FN from bronchitis and normal subjects.

Main Methods:

  • Purification of fibronectin (FN) from plasma of CF patients, bronchitis patients, and normal subjects.
  • Enzyme-linked immunosorbent assay (ELISA) technique to assess bacterial binding to FN.
  • Polyacrylamide gel electrophoresis (PAGE) to analyze peptide migration patterns after proteinase hydrolysis.
  • Quantification of total sugar content in FN samples.

Main Results:

  • A significant decrease in the binding of CF-derived FN to S. aureus and Streptococcus pyogenes was observed in all seven CF patients studied.
  • Marked alterations in peptide migration patterns were noted in CF FN compared to normal FN after enzymatic hydrolysis.
  • Variations in total sugar content (increase or decrease) were found in CF FN.

Conclusions:

  • Fibronectin from cystic fibrosis patients exhibits reduced binding affinity for key respiratory pathogens.
  • Structural and compositional modifications in CF fibronectin may play a role in the susceptibility to recurrent bacterial lung infections.
  • These findings suggest fibronectin alterations as a potential factor contributing to the pathophysiology of cystic fibrosis lung disease.

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