Proton-chloride exchanger 5

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Proton-chloride exchanger 5
Properties of human protein
Mass / length primary structure 746 amino acids
Secondary to quaternary structure multipass membrane protein
Identifier
Gene name CLCN5
External IDs
Transporter classification
TCDB 2.A.49.2.2
designation Chloride channel
Occurrence
Homology family Chloride channels
Parent taxon Fungi / Metazoa

The proton chloride exchanger 5 (also: chloride transporter ClC-5 ) is a protein in the cells of animals and fungi. It is found in the cell membrane and inside the cell in the membrane of the Golgi apparatus and of endosomes , where it moves chloride ions from the inside of the respective cell compartment to the outside and at the same time transports protons inside. The transport protein is thus responsible, for example, for acidifying the endosome lumen. ClC-5 plays a central role in the reabsorption of proteins in the kidneys through endocytosis .

Rare mutations in CLCN5 - gene may have as well as oversupply of ClC-5 function result both deficiency. ClC-5 deficiency can lead to several clinical pictures, which are summarized in the so-called Dent syndrome and are associated with proteinuria , calcium kidney stones , hypophosphataemia , rickets and kidney failure. The defects in renal reabsorption are due to the inability of proximal tubular cells to reabsorb the transport proteins megalin and cubilin .

The transport equation is

2 anion (inside) + H + (outside) = 2 anion (outside) + H + (inside)

In addition to chloride, nitrates , bromides and iodides are also accepted as anions with an affinity of NO 3 - > Cl - > Br - > I - .

Individual evidence

  1. ↑ a b UniProt P51795
  2. ↑ TJ Jentsch: CLC chloride channels and transporters: from genes to protein structure, pathology and physiology. In: Critical Reviews in Biochemistry and Molecular Biology. Volume 43, Number 1, 2008 Jan-Feb, pp. 3-36, doi : 10.1080 / 10409230701829110 , PMID 18307107 (review).
  3. ↑ EI Christensen, O. Devuyst et al. a .: Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules. In: Proceedings of the National Academy of Sciences . Volume 100, number 14, July 2003, pp. 8472-8477, doi : 10.1073 / pnas.1432873100 , PMID 12815097 , PMC 166253 (free full text).
  4. ↑ TCDB : 2.A.49

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