chloride channel protein ClC-Kb; chloride channel Kb; ClC-K2 (CLCNKB)

Function

• voltage-gated chloride channel
• chloride channels have several functions including
  • regulation of cell volume
  • membrane potential stabilization
  • signal transduction
  • transepithelial transport
• may be important in urinary concentrating mechanisms
• interacts with barttin (BSND)
• forms heteromers with BSND in the thick ascending limb of Henle & more distal segments

Structure

• belongs to the chloride channel (TC 2.A.49) family
• contains 2 CBS domains

Compartment

membrane

Expression

expressed predominantly in the kidney

Pathology

• defects in CLCNKB are the cause of Bartter syndrome type 3

Notes

• compared with CLCNKA/BSND, CLCNKB/BSND is more sensitive to pH & less responsive to Ca+2

More general terms

• Cl- channel
• glycoprotein
• transmembrane 12 protein

Database

• UniProt: http://www.uniprot.org/uniprot/P51801.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1188
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1188
• OMIM: https://mirror.omim.org/entry/602023
• OMIM: https://mirror.omim.org/entry/607364