title: Reduced absorption and enhanced synthesis of cholesterol in patients with cystic fibrosis: a preliminary study of plasma sterols.
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.
authors.forename: Monica
authors.initials: M
authors.lastname: Gelzo
authors.forename: Concetta
authors.initials: C
authors.lastname: Sica
authors.forename: Ausilia
authors.initials: A
authors.lastname: Elce
authors.forename: Antonio
authors.initials: A
authors.lastname: Dello Russo
authors.forename: Paola
authors.initials: P
authors.lastname: Iacotucci
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Carnovale
authors.forename: Valeria
authors.initials: V
authors.lastname: Raia
authors.forename: Donatello
authors.initials: D
authors.lastname: Salvatore
authors.forename: Gaetano
authors.initials: G
authors.lastname: Corso
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Castaldo
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000031:analogs & derivatives / Q000096:biosynthesis* / Q000097:blood; D002849:Chromatography, Gas; D003550:Cystic Fibrosis / Q000097:blood*; D005260:Female; D006801:Humans; D007223:Infant; D007408:Intestinal Absorption; D008297:Male; D008875:Middle Aged; D010840:Phytosterols / Q000097:blood; D013261:Sterols / Q000097:blood*; D055815:Young Adult
chemical_list: D015415:Biomarkers; D010840:Phytosterols; D013261:Sterols; C021273:campesterol; D002784:Cholesterol