Cystic fibrosis transmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone

J Cyst Fibros. 2010 Jan;9(1):69-72. doi: 10.1016/j.jcf.2009.11.005. Epub 2009 Dec 14.

Abstract

Bone loss is an important clinical issue in patients with cystic fibrosis (CF). Whether the cystic fibrosis transmembrane conductance regulator (CFTR) plays a direct role in bone cell function is yet unknown. In this study, we provide evidence that inhibition of CFTR-Cl(-) channel function results in a significant decrease of osteoprotegerin (OPG) secretion accompanied with a concomitant increase of prostaglandin (PG) E(2) secretion of primary human osteoblast cultures (n=5). Our data therefore suggest that in bone cells of CF patients, the loss of CFTR activity may result in an increased inflammation-driven bone resorption (through both the reduced OPG and increased PGE(2) production), and thus might contribute to the early bone loss reported in young children with CF.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Bone Resorption / etiology*
  • Bone Resorption / physiopathology
  • Cells, Cultured
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Dinoprostone / metabolism*
  • Female
  • Humans
  • Middle Aged
  • Osteoblasts / cytology
  • Osteoblasts / physiology
  • Osteoprotegerin / metabolism*
  • RNA, Messenger / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction

Substances

  • CFTR protein, human
  • Osteoprotegerin
  • RNA, Messenger
  • TNFRSF11B protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Dinoprostone