Characterization of acid flux in osteoclasts from patients harboring a G215R mutation in ClC-7

Biochem Biophys Res Commun. 2009 Jan 23;378(4):804-9. doi: 10.1016/j.bbrc.2008.11.145. Epub 2008 Dec 12.

Abstract

The chloride-proton antiporter ClC-7 has been speculated to be involved in acidification of the lysosomes and the resorption lacunae in osteoclasts; however, neither direct measurements of chloride transport nor acidification have been performed. Human osteoclasts harboring a dominant negative mutation in ClC-7 (G215R) were isolated, and used these to investigate bone resorption measured by CTX-I, calcium release and pit scoring. The actin cytoskeleton of the osteoclasts was also investigated. ClC-7 enriched membranes from the osteoclasts were isolated, and used to test acidification rates in the presence of a V-ATPase and a chloride channel inhibitor, using a H(+) and Cl(-) driven approach. Finally, acidification rates in ClC-7 enriched membranes from ADOII osteoclasts and their corresponding controls were compared. Resorption by the G215R osteoclasts was reduced by 60% when measured by both CTX-I, calcium release, and pit area when comparing to age and sex matched controls. In addition, the ADOII osteoclasts showed no differences in actin ring formation. Finally, V-ATPase and chloride channel inhibitors completely abrogated the H(+) and Cl(-) driven acidification. Finally, the acid influx was reduced by maximally 50% in the ClC-7 deficient membrane fractions when comparing to controls. These data demonstrate that ClC-7 is essential for bone resorption, via its role in acidification of the lysosomes and resorption lacunae in osteoclasts.

MeSH terms

  • Acids / metabolism
  • Arginine / genetics
  • Bone Resorption / genetics
  • Bone Resorption / metabolism*
  • Calcium / metabolism
  • Chloride Channels / antagonists & inhibitors
  • Chloride Channels / genetics
  • Chloride Channels / physiology*
  • Genes, Dominant
  • Glycine / genetics
  • Humans
  • Hydrogen-Ion Concentration
  • Lysosomes / metabolism*
  • Mutation
  • Osteoclasts / metabolism*
  • Osteoclasts / ultrastructure
  • Vacuolar Proton-Translocating ATPases / antagonists & inhibitors
  • Vacuolar Proton-Translocating ATPases / metabolism

Substances

  • Acids
  • CLCN7 protein, human
  • Chloride Channels
  • Arginine
  • Vacuolar Proton-Translocating ATPases
  • Calcium
  • Glycine