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Identification
HMDB Protein ID HMDBP08183
Secondary Accession Numbers
  • 13894
Name Chloride channel protein ClC-Kb
Synonyms
  1. Chloride channel Kb
  2. ClC-K2
Gene Name CLCNKB
Protein Type Unknown
Biological Properties
General Function Involved in ion channel activity
Specific Function Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms
Pathways Not Available
Reactions Not Available
GO Classification
Component
membrane
cell part
Function
voltage-gated chloride channel activity
anion channel activity
chloride channel activity
transmembrane transporter activity
substrate-specific transmembrane transporter activity
ion transmembrane transporter activity
transporter activity
ion channel activity
Process
establishment of localization
transport
chloride transport
transmembrane transport
anion transport
inorganic anion transport
ion transport
Cellular Location
  1. Cell membrane
  2. Multi-pass membrane protein
Gene Properties
Chromosome Location Chromosome:1
Locus 1p36
SNPs CLCNKB
Gene Sequence
>2064 bp
ATGGAGGAGTTTGTGGGGCTGCGTGAAGGCTCCTCAGGGAACCCTGTGACTCTGCAGGAG
CTGTGGGGCCCCTGTCCCCTCATCCGCCGAGGCATCCGAGGTGGCCTGGAGTGGCTGAAG
CAGAAGCTCTTCCGCCTGGGCGAGGACTGGTACTTCCTGATGACCCTCGGGGTGCTCATG
GCCCTGGTCAGCTGTGCCATGGACTTGGCTGTTGAGAGTGTGGTCCGAGCGCACCAGTGG
CTGTACAGGGAGATTGGGGACAGCCACCTGCTCCGGTATCTCTCCTGGACTGTGTACCCT
GTGGCCCTCGTCTCTTTCTCTTCGGGCTTCTCTCAGAGCATCACACCCTCCTCTGGAGGT
TCTGGAATCCCGGAGGTGAAGACCATGTTGGCGGGTGTGGTCTTGGAGGACTACCTGGAT
ATCAAGAACTTTGGGGCCAAAGTGGTGGGCCTCTCCTGCACCCTGGCCTGTGGCAGCACC
CTCTTCCTCGGCAAAGTGGGCCCTTTCGTGCACCTGTCTGTGATGATGGCTGCCTACCTG
GGCCGTGTGCGCACCACGACCATCGGGGAGCCTGAGAACAAGAGCAAGCAAAACGAAATG
CTGGTGGCAGCGGCGGCAGTGGGCGTGGCCACAGTCTTTGGCGCTCCCTTCAGCGGCGTC
CTGTTCAGCATCGAGGTCATGTCTTCCCACTTCTCTGTCTGGGATTACTGGAGGGGCTTC
TTTGCGGCCACCTGCGGGGCCTTCATGTTCCGGCTCCTGGCGGTCTTCAACAGCGAGCAG
GAGACCATCACCTCCCTCTACAAGACCAGTTTCCGGGTGGACGTTCCCTTCGACCTGCCT
GAGATCTTCTTTTTTGTGGTGCTGGGGGGTCTCTGCGGCATCCTGGGCAGCGCTTACCTC
TTCTGTCAGCGAATCTTCTTTGGCTTCATCAGGAACAATAGGTTCAGCTCCAAACTGCTG
GCCACCAGCAAGCCTGTGTACTCCGCTCTGGCCACCTTGGTTCTCGCCTCCATCACCTAC
CCACCCAGCGCCGGCCGCTTCCTAGCTTCTCGGCTGTCCATGAAGCAGCATCTGGACTCG
CTGTTCGACAACCACTCCTGGGCGCTGATGACCCAGAACTCCAGCCCACCCTGGCCCGAG
GAGCTCGACCCCCAGCACCTGTGGTGGGAATGGTACCACCCGCGGTTCACCATCTTTGGG
ACCCTTGCCTTCTTCCTGGTTATGAAGTTCTGGATGCTGATTCTGGCCACCACCATCCCC
ATGCCTGCCGGGTACTTCATGCCCATCTTTGTCTATGGAGCTGCTATCGGGCGCCTCTTT
GGGGAGACTCTCTCTTTTATCTTCCCTGAGGGCATCGTGGCTGGAGGGATCACCAATCCC
ATCATGCCAGGGGGGTATGCTCTGGCAGGGGCTGCAGCCTTCTCAGGGGCTGTGACCCAC
ACCATCTCCACGGCGCTGCTGGCCTTCGAGGTGACCGGCCAGATAGTGCATGCACTGCCC
GTGCTGATGGCGGTGCTGGCAGCCAACGCCATTGCACAGAGCTGCCAGCCCTCCTTCTAT
GATGGCACCGTCATTGTCAAGAAGCTGCCATACCTGCCACGGATTCTGGGCCGCAACATC
GGTTCCCACCGCGTGAGGGTGGAGCACTTCATGAACCACAGCATCACCACACTGGCCAAG
GACACGCCACTGGAGGAGGTGGTCAAGGTTGTGACCTCCACAGACGTGGCCGAGTATCCC
TTGGTGGAGAGCACAGAGTCCCAGATCCTGGTGGGCATAGTGCGAAGGGCCCAGCTGGTG
CAGGCCCTGAAGGCTGAGCCTCCTTCCTGGGCTCCTGGACACCAGCAGTGTCTCCAGGAC
ATCTTGGCTGCAGGCTGCCCCACAGAACCAGTGACCCTGAAGCTGTCCCCAGAGACTTCC
CTGCATGAGGCACACAACCTCTTTGAGCTGTTGAACCTTCATTCCCTCTTTGTGACGTCG
CGGGGCAGAGCTGTGGGCTGCGTGTCCTGGGTGGAGATGAAGAAAGCAATTTCCAACCTG
ACAAATCCGCCAGCCCCAAAGTGA
Protein Properties
Number of Residues 687
Molecular Weight 75445.3
Theoretical pI 8.07
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • 51-71
  • 94-114
  • 161-181
  • 202-222
  • 236-256
  • 282-302
  • 325-345
  • 396-416
  • 417-437
  • 452-472
  • 486-506
Protein Sequence
>Chloride channel protein ClC-Kb
MEEFVGLREGSSGNPVTLQELWGPCPRIRRGIRGGLEWLKQKLFRLGEDWYFLMTLGVLM
ALVSCAMDLAVESVVRAHQWLYREIGDSHLLRYLSWTVYPVALVSFSSGFSQSITPSSGG
SGIPEVKTMLAGVVLEDYLDIKNFGAKVVGLSCTLACGSTLFLGKVGPFVHLSVMMAAYL
GRVRTTTIGEPENKSKQNEMLVAAAAVGVATVFAAPFSGVLFSIEVMSSHFSVWDYWRGF
FAATCGAFMFRLLAVFNSEQETITSLYKTSFRVDVPFDLPEIFFFVALGGLCGILGSAYL
FCQRIFFGFIRNNRFSSKLLATSKPVYSALATLVLASITYPPSAGRFLASRLSMKQHLDS
LFDNHSWALMTQNSSPPWPEELDPQHLWWEWYHPRFTIFGTLAFFLVMKFWMLILATTIP
MPAGYFMPIFVYGAAIGRLFGETLSFIFPEGIVAGGITNPIMPGGYALAGAAAFSGAVTH
TISTALLAFEVTGQIVHALPVLMAVLAANAIAQSCQPSFYDGTVIVKKLPYLPRILGRNI
GSHRVRVEHFMNHSITTLAKDMPLEEVVKVVTSTDVAKYPLVESTESQILVGIVRRAQLV
QALKAEPPSWAPGHQQCLQDILAAGCPTEPVTLKLSPETSLHEAHNLFELLNLHSLFVTS
RGRAVGCVSWVEMKKAISNLTNPPAPK
GenBank ID Protein 521074
UniProtKB/Swiss-Prot ID P51801
UniProtKB/Swiss-Prot Entry Name CLCKB_HUMAN
PDB IDs Not Available
GenBank Gene ID Z30644
GeneCard ID CLCNKB
GenAtlas ID CLCNKB
HGNC ID HGNC:2027
References
General References
  1. Gregory SG, Barlow KF, McLay KE, Kaul R, Swarbreck D, Dunham A, Scott CE, Howe KL, Woodfine K, Spencer CC, Jones MC, Gillson C, Searle S, Zhou Y, Kokocinski F, McDonald L, Evans R, Phillips K, Atkinson A, Cooper R, Jones C, Hall RE, Andrews TD, Lloyd C, Ainscough R, Almeida JP, Ambrose KD, Anderson F, Andrew RW, Ashwell RI, Aubin K, Babbage AK, Bagguley CL, Bailey J, Beasley H, Bethel G, Bird CP, Bray-Allen S, Brown JY, Brown AJ, Buckley D, Burton J, Bye J, Carder C, Chapman JC, Clark SY, Clarke G, Clee C, Cobley V, Collier RE, Corby N, Coville GJ, Davies J, Deadman R, Dunn M, Earthrowl M, Ellington AG, Errington H, Frankish A, Frankland J, French L, Garner P, Garnett J, Gay L, Ghori MR, Gibson R, Gilby LM, Gillett W, Glithero RJ, Grafham DV, Griffiths C, Griffiths-Jones S, Grocock R, Hammond S, Harrison ES, Hart E, Haugen E, Heath PD, Holmes S, Holt K, Howden PJ, Hunt AR, Hunt SE, Hunter G, Isherwood J, James R, Johnson C, Johnson D, Joy A, Kay M, Kershaw JK, Kibukawa M, Kimberley AM, King A, Knights AJ, Lad H, Laird G, Lawlor S, Leongamornlert DA, Lloyd DM, Loveland J, Lovell J, Lush MJ, Lyne R, Martin S, Mashreghi-Mohammadi M, Matthews L, Matthews NS, McLaren S, Milne S, Mistry S, Moore MJ, Nickerson T, O'Dell CN, Oliver K, Palmeiri A, Palmer SA, Parker A, Patel D, Pearce AV, Peck AI, Pelan S, Phelps K, Phillimore BJ, Plumb R, Rajan J, Raymond C, Rouse G, Saenphimmachak C, Sehra HK, Sheridan E, Shownkeen R, Sims S, Skuce CD, Smith M, Steward C, Subramanian S, Sycamore N, Tracey A, Tromans A, Van Helmond Z, Wall M, Wallis JM, White S, Whitehead SL, Wilkinson JE, Willey DL, Williams H, Wilming L, Wray PW, Wu Z, Coulson A, Vaudin M, Sulston JE, Durbin R, Hubbard T, Wooster R, Dunham I, Carter NP, McVean G, Ross MT, Harrow J, Olson MV, Beck S, Rogers J, Bentley DR, Banerjee R, Bryant SP, Burford DC, Burrill WD, Clegg SM, Dhami P, Dovey O, Faulkner LM, Gribble SM, Langford CF, Pandian RD, Porter KM, Prigmore E: The DNA sequence and biological annotation of human chromosome 1. Nature. 2006 May 18;441(7091):315-21. [PubMed:16710414 ]
  2. Estevez R, Boettger T, Stein V, Birkenhager R, Otto E, Hildebrandt F, Jentsch TJ: Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion. Nature. 2001 Nov 29;414(6863):558-61. [PubMed:11734858 ]
  3. Kieferle S, Fong P, Bens M, Vandewalle A, Jentsch TJ: Two highly homologous members of the ClC chloride channel family in both rat and human kidney. Proc Natl Acad Sci U S A. 1994 Jul 19;91(15):6943-7. [PubMed:8041726 ]
  4. Nozu K, Inagaki T, Fu XJ, Nozu Y, Kaito H, Kanda K, Sekine T, Igarashi T, Nakanishi K, Yoshikawa N, Iijima K, Matsuo M: Molecular analysis of digenic inheritance in Bartter syndrome with sensorineural deafness. J Med Genet. 2008 Mar;45(3):182-6. doi: 10.1136/jmg.2007.052944. [PubMed:18310267 ]
  5. Schlingmann KP, Konrad M, Jeck N, Waldegger P, Reinalter SC, Holder M, Seyberth HW, Waldegger S: Salt wasting and deafness resulting from mutations in two chloride channels. N Engl J Med. 2004 Mar 25;350(13):1314-9. [PubMed:15044642 ]
  6. Takeuchi Y, Uchida S, Marumo F, Sasaki S: Cloning, tissue distribution, and intrarenal localization of ClC chloride channels in human kidney. Kidney Int. 1995 Nov;48(5):1497-503. [PubMed:8544406 ]
  7. Simon DB, Bindra RS, Mansfield TA, Nelson-Williams C, Mendonca E, Stone R, Schurman S, Nayir A, Alpay H, Bakkaloglu A, Rodriguez-Soriano J, Morales JM, Sanjad SA, Taylor CM, Pilz D, Brem A, Trachtman H, Griswold W, Richard GA, John E, Lifton RP: Mutations in the chloride channel gene, CLCNKB, cause Bartter's syndrome type III. Nat Genet. 1997 Oct;17(2):171-8. [PubMed:9326936 ]