ATP-sensitive inward rectifier potassium channel 10

Details

Name
ATP-sensitive inward rectifier potassium channel 10
Kind
protein
Synonyms
  • ATP-dependent inwardly rectifying potassium channel Kir4.1
  • Inward rectifier K(+) channel Kir1.2
  • Potassium channel, inwardly rectifying subfamily J member 10
Gene Name
KCNJ10
UniProtKB Entry
P78508Swiss-Prot
Organism
Humans
NCBI Taxonomy ID
9606
Amino acid sequence
>lcl|BSEQ0008683|ATP-sensitive inward rectifier potassium channel 10
MTSVAKVYYSQTTQTESRPLMGPGIRRRRVLTKDGRSNVRMEHIADKRFLYLKDLWTTFI
DMQWRYKLLLFSATFAGTWFLFGVVWYLVAVAHGDLLELDPPANHTPCVVQVHTLTGAFL
FSLESQTTIGYGFRYISEECPLAIVLLIAQLVLTTILEIFITGTFLAKIARPKKRAETIR
FSQHAVVASHNGKPCLMIRVANMRKSLLIGCQVTGKLLQTHQTKEGENIRLNQVNVTFQV
DTASDSPFLILPLTFYHVVDETSPLKDLPLRSGEGDFELVLILSGTVESTSATCQVRTSY
LPEEILWGYEFTPAISLSASGKYIADFSLFDQVVKVASPSGLRDSTVRYGDPEKLKLEES
LREQAEKEGSALSVRISNV
Number of residues
379
Molecular Weight
42507.71
Theoretical pI
Not Available
GO Classification
Processes
cellular response to potassium ion / non-motile cilium assembly / potassium ion import across plasma membrane / regulation of monoatomic ion transmembrane transport
Components
astrocyte projection / cell body / ciliary base / monoatomic ion channel complex
General Function
May be responsible for potassium buffering action of glial cells in the brain. Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it. Their voltage dependence is regulated by the concentration of extracellular potassium; as external potassium is raised, the voltage range of the channel opening shifts to more positive voltages. The inward rectification is mainly due to the blockage of outward current by internal magnesium. Can be blocked by extracellular barium and cesium (By similarity). In the kidney, together with KCNJ16, mediates basolateral K(+) recycling in distal tubules; this process is critical for Na(+) reabsorption at the tubules
Specific Function
Atp binding
Pfam Domain Function
Signal Regions
Not Available
Transmembrane Regions
65-89 143-164
Cellular Location
Membrane
Gene sequence
>lcl|BSEQ0019642|ATP-sensitive inward rectifier potassium channel 10 (KCNJ10)
ATGACGTCAGTTGCCAAGGTGTATTACAGTCAGACCACTCAGACAGAAAGCCGGCCCCTA
ATGGGCCCAGGGATACGACGGCGGAGAGTCCTGACAAAAGATGGTCGCAGCAACGTGAGA
ATGGAGCACATTGCCGACAAGCGCTTCCTCTACCTCAAGGACCTGTGGACAACCTTCATT
GACATGCAGTGGCGCTACAAGCTTCTGCTCTTCTCTGCGACCTTTGCAGGCACATGGTTC
CTCTTTGGCGTGGTGTGGTATCTGGTAGCTGTGGCACATGGGGACCTGCTGGAGCTGGAC
CCCCCGGCCAACCACACCCCCTGTGTGGTACAGGTGCACACACTCACTGGAGCCTTCCTC
TTCTCCCTTGAATCCCAAACCACCATTGGCTATGGCTTCCGCTACATCAGTGAGGAATGT
CCACTGGCCATTGTGCTTCTTATTGCCCAGCTGGTGCTCACCACCATCCTGGAAATCTTC
ATCACAGGTACCTTCCTGGCGAAGATTGCCCGGCCCAAGAAGCGGGCTGAGACCATTCGT
TTCAGCCAGCATGCAGTTGTGGCCTCCCACAATGGCAAGCCCTGCCTCATGATCCGAGTT
GCCAATATGCGCAAAAGCCTCCTCATTGGCTGCCAGGTGACAGGAAAACTGCTTCAGACC
CACCAAACCAAGGAAGGGGAGAACATCCGGCTCAACCAGGTCAATGTGACTTTCCAAGTA
GACACAGCCTCTGACAGCCCCTTCCTTATTCTACCCCTTACCTTCTATCATGTGGTAGAT
GAGACCAGTCCCTTGAAAGATCTCCCTCTTCGCAGTGGTGAGGGTGACTTTGAGCTGGTG
CTGATCCTAAGTGGGACAGTGGAGTCCACCAGTGCCACCTGTCAGGTGCGCACTTCCTAC
CTGCCAGAGGAGATCCTTTGGGGCTACGAGTTCACACCTGCCATCTCACTGTCAGCCAGT
GGTAAATACATAGCTGACTTTAGCCTTTTTGACCAAGTTGTGAAAGTGGCCTCTCCTAGT
GGCCTCCGTGACAGCACTGTACGCTACGGAGACCCTGAAAAGCTCAAGTTGGAGGAGTCA
TTAAGGGAGCAAGCTGAGAAGGAGGGCAGTGCCCTTAGTGTGCGCATCAGCAATGTCTGA
Chromosome Location
1
Locus
1q23.2
External Identifiers
ResourceLink
UniProtKB IDP78508
UniProtKB Entry NameKCJ10_HUMAN
GeneCard IDKCNJ10
HGNC IDHGNC:6256
KEGG IDhsa:3766
IUPHAR/Guide To Pharmacology ID438
NCBI Gene ID3766
General References
  1. Shuck ME, Piser TM, Bock JH, Slightom JL, Lee KS, Bienkowski MJ: Cloning and characterization of two K+ inward rectifier (Kir) 1.1 potassium channel homologs from human kidney (Kir1.2 and Kir1.3). J Biol Chem. 1997 Jan 3;272(1):586-93. [Article]
  2. Schoots O, Wilson JM, Ethier N, Bigras E, Hebert TE, Van Tol HH: Co-expression of human Kir3 subunits can yield channels with different functional properties. Cell Signal. 1999 Dec;11(12):871-83. [Article]
  3. 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. [Article]
  4. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  5. Bockenhauer D, Feather S, Stanescu HC, Bandulik S, Zdebik AA, Reichold M, Tobin J, Lieberer E, Sterner C, Landoure G, Arora R, Sirimanna T, Thompson D, Cross JH, van't Hoff W, Al Masri O, Tullus K, Yeung S, Anikster Y, Klootwijk E, Hubank M, Dillon MJ, Heitzmann D, Arcos-Burgos M, Knepper MA, Dobbie A, Gahl WA, Warth R, Sheridan E, Kleta R: Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations. N Engl J Med. 2009 May 7;360(19):1960-70. doi: 10.1056/NEJMoa0810276. [Article]
  6. Scholl UI, Choi M, Liu T, Ramaekers VT, Hausler MG, Grimmer J, Tobe SW, Farhi A, Nelson-Williams C, Lifton RP: Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SeSAME syndrome) caused by mutations in KCNJ10. Proc Natl Acad Sci U S A. 2009 Apr 7;106(14):5842-7. doi: 10.1073/pnas.0901749106. Epub 2009 Mar 16. [Article]
  7. Lemke JR, Riesch E, Scheurenbrand T, Schubach M, Wilhelm C, Steiner I, Hansen J, Courage C, Gallati S, Burki S, Strozzi S, Simonetti BG, Grunt S, Steinlin M, Alber M, Wolff M, Klopstock T, Prott EC, Lorenz R, Spaich C, Rona S, Lakshminarasimhan M, Kroll J, Dorn T, Kramer G, Synofzik M, Becker F, Weber YG, Lerche H, Bohm D, Biskup S: Targeted next generation sequencing as a diagnostic tool in epileptic disorders. Epilepsia. 2012 Aug;53(8):1387-98. doi: 10.1111/j.1528-1167.2012.03516.x. Epub 2012 May 21. [Article]
  8. Tanemoto M, Abe T, Uchida S, Kawahara K: Mislocalization of K+ channels causes the renal salt wasting in EAST/SeSAME syndrome. FEBS Lett. 2014 Mar 18;588(6):899-905. doi: 10.1016/j.febslet.2014.02.024. Epub 2014 Feb 20. [Article]
  9. Zhou XL, He LX, Yu LJ, Wang Y, Wang XJ, Wang ED, Yang T: Mutations in KARS cause early-onset hearing loss and leukoencephalopathy: Potential pathogenic mechanism. Hum Mutat. 2017 Dec;38(12):1740-1750. doi: 10.1002/humu.23335. Epub 2017 Sep 29. [Article]

Associated Data

Drug Relations
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Tolazamideapproved, investigationalyestargetblockerDetails
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