Reduced absorption and enhanced synthesis of cholesterol in patients with cystic fibrosis: A preliminary study of plasma sterols

Monica Gelzo, Concetta Sica, Ausilia Elce, Antonio Dello Russo, Paola Iacotucci, Vincenzo Carnovale, Valeria Raia, Donatello Salvatore, Gaetano Corso, Giuseppe Castaldo

Research output: Contribution to journalArticle

Abstract

Background: Low cholesterol is typically observed in the plasma of patients with cystic fibrosis (CF) contrasting with the subcellular accumulation of cholesterol demonstrated in CF cells and in mice models. However, the homeostasis of cholesterol has not been well investigated in patients with CF. Methods: We studied the plasma of 26 patients with CF and 33 unaffected controls campesterol and β-sitosterol as markers of intestinal absorption and lathosterol as a marker of de novo cholesterol biosynthesis by gas chromatography (GC-FID and GC-MS). Results: Plasma campesterol and β-sitosterol results were significantly (p = 0.01) lower while plasma lathosterol was significantly higher (p = 0.001) in patients with CF as compared to control subjects. Plasma cholesterol results were significantly lower (p = 0.01) in CF patients. Conclusions: Our data suggest that the impaired intestinal absorption of exogenous sterols in patients with CF stimulates the endogenous synthesis of cholesterol, but the levels of total cholesterol in plasma remain lower. This may be due to the CFTR dysfunction that reduces cholesterol blood excretion causing the accumulation of cholesterol in liver cells and in other tissues contributing to trigger CF chronic inflammation.

Original languageEnglish
Pages (from-to)1461-1466
Number of pages6
JournalClinical Chemistry and Laboratory Medicine
Volume54
Issue number9
DOIs
Publication statusPublished - Sep 1 2016

Keywords

  • cholesterol metabolism
  • cystic fibrosis
  • gas chromatography

ASJC Scopus subject areas

  • Medicine(all)
  • Clinical Biochemistry
  • Biochemistry, medical

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    Gelzo, M., Sica, C., Elce, A., Dello Russo, A., Iacotucci, P., Carnovale, V., Raia, V., Salvatore, D., Corso, G., & Castaldo, G. (2016). Reduced absorption and enhanced synthesis of cholesterol in patients with cystic fibrosis: A preliminary study of plasma sterols. Clinical Chemistry and Laboratory Medicine, 54(9), 1461-1466. https://doi.org/10.1515/cclm-2015-1151