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Chloride channel, voltage-sensitive 5
Protein CLCN5 PDB 2j9l.png
PDB rendering based on 2j9l.
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM300008 MGI99486 HomoloGene73872 IUPHAR: 704 GeneCards: CLCN5 Gene
RNA expression pattern
PBB GE CLCN5 206704 at tn.png
More reference expression data
Species Human Mouse
Entrez 1184 12728
Ensembl ENSG00000171365 ENSMUSG00000004317
UniProt P51795 Q9WVD4
RefSeq (mRNA) NM_000084 NM_001243762
RefSeq (protein) NP_000075 NP_001230691
Location (UCSC) Chr X:
49.92 – 50.1 Mb
Chr X:
7.15 – 7.32 Mb
PubMed search [1] [2]

H(+)/Cl(-) exchange transporter 5 is a protein that in humans is encoded by the CLCN5 gene.[1][2]

This gene encodes a member of the ClC family of chloride ion channels and ion transporters. Mutations in this gene have been found in Dent's Disease and renal tubular disorders complicated by nephrolithiasis.[3] Although a member of a family of chloride channels, the CLCN5 protein allows movement of protons in the opposite direction of Cl(-), thus functioning as an antiporter. [4]

See also[edit]


  1. ^ Fisher SE, Black GC, Lloyd SE, Hatchwell E, Wrong O, Thakker RV, Craig IW (Apr 1995). "Isolation and partial characterization of a chloride channel gene which is expressed in kidney and is a candidate for Dent's disease (an X-linked hereditary nephrolithiasis)". Hum Mol Genet 3 (11): 2053–9. PMID 7874126. 
  2. ^ Pook MA, Wrong O, Wooding C, Norden AG, Feest TG, Thakker RV (Mar 1994). "Dent's disease, a renal Fanconi syndrome with nephrocalcinosis and kidney stones, is associated with a microdeletion involving DXS255 and maps to Xp11.22". Hum Mol Genet 2 (12): 2129–34. doi:10.1093/hmg/2.12.2129. PMID 8111383. 
  3. ^ "Entrez Gene: CLCN5 chloride channel 5 (nephrolithiasis 2, X-linked, Dent disease)". 
  4. ^ Picollo A, Pusch M (2005). "Chloride/proton antiporter activity of mammalian CLC proteins ClC-4 and ClC-5.". Nature 436 (7049): 420–3. doi:10.1038/nature03720. PMID 16034421. 

Further reading[edit]

External links[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.