Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins

Nature. 2005 Jul 21;436(7049):424-7. doi: 10.1038/nature03860.

Abstract

Eukaryotic members of the CLC gene family function as plasma membrane chloride channels, or may provide neutralizing anion currents for V-type H(+)-ATPases that acidify compartments of the endosomal/lysosomal pathway. Loss-of-function mutations in the endosomal protein ClC-5 impair renal endocytosis and lead to kidney stones, whereas loss of function of the endosomal/lysosomal protein ClC-7 entails osteopetrosis and lysosomal storage disease. Vesicular CLCs have been thought to be Cl- channels, in particular because ClC-4 and ClC-5 mediate plasma membrane Cl- currents upon heterologous expression. Here we show that these two mainly endosomal CLC proteins instead function as electrogenic Cl-/H+ exchangers (also called antiporters), resembling the transport activity of the bacterial protein ClC-e1, the crystal structure of which has already been determined. Neutralization of a critical glutamate residue not only abolished the steep voltage-dependence of transport, but also eliminated the coupling of anion flux to proton counter-transport. ClC-4 and ClC-5 may still compensate the charge accumulation by endosomal proton pumps, but are expected to couple directly vesicular pH gradients to Cl- gradients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antiporters / genetics
  • Antiporters / metabolism*
  • Bacterial Proteins / genetics
  • Bacterial Proteins / metabolism
  • Cell Line
  • Chloride Channels / genetics
  • Chloride Channels / metabolism*
  • Chlorides / metabolism*
  • Electric Conductivity
  • Endosomes / metabolism*
  • Humans
  • Hydrogen-Ion Concentration
  • Ion Channel Gating*
  • Ion Transport
  • Membrane Potentials
  • Mutation, Missense / genetics
  • Oocytes / metabolism
  • Patch-Clamp Techniques
  • Protons*
  • Torpedo
  • Xenopus

Substances

  • Antiporters
  • Bacterial Proteins
  • CLC-5 chloride channel
  • Chloride Channels
  • Chlorides
  • Protons