NameATP-sensitive inward rectifier potassium channel 8
Synonyms
  • Inward rectifier K(+) channel Kir6.1
  • Potassium channel, inwardly rectifying subfamily J member 8
  • uKATP-1
Gene NameKCNJ8
OrganismHuman
Amino acid sequence
>lcl|BSEQ0019285|ATP-sensitive inward rectifier potassium channel 8
MLARKSIIPEEYVLARIAAENLRKPRIRDRLPKARFIAKSGACNLAHKNIREQGRFLQDI
FTTLVDLKWRHTLVIFTMSFLCSWLLFAIMWWLVAFAHGDIYAYMEKSGMEKSGLESTVC
VTNVRSFTSAFLFSIEVQVTIGFGGRMMTEECPLAITVLILQNIVGLIINAVMLGCIFMK
TAQAHRRAETLIFSRHAVIAVRNGKLCFMFRVGDLRKSMIISASVRIQVVKKTTTPEGEV
VPIHQLDIPVDNPIESNNIFLVAPLIICHVIDKRSPLYDISATDLANQDLEVIVILEGVV
ETTGITTQARTSYIAEEIQWGHRFVSIVTEEEGVYSVDYSKFGNTVKVAAPRCSARELDE
KPSILIQTLQKSELSHQNSLRKRNSMRRNNSMRRNNSIRRNNSSLMVPKVQFMTPEGNQN
TSES
Number of residues424
Molecular Weight47967.455
Theoretical pI9.7
GO Classification
Functions
  • inward rectifier potassium channel activity
  • ATP-activated inward rectifier potassium channel activity
  • ATP binding
Processes
  • potassium ion transport
  • synaptic transmission
  • kidney development
  • potassium ion import
  • response to pH
  • regulation of ion transmembrane transport
  • vasodilation
Components
  • plasma membrane
  • voltage-gated potassium channel complex
  • integral component of plasma membrane
  • sarcolemma
  • ATP-sensitive potassium channel complex
  • mitochondrion
General FunctionInward rectifier potassium channel activity
Specific FunctionThis potassium channel is controlled by G proteins. 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 external barium (By similarity).
Pfam Domain Function
Transmembrane Regions70-94 155-176
GenBank Protein IDNot Available
UniProtKB IDQ15842
UniProtKB Entry NameKCNJ8_HUMAN
Cellular LocationMembrane
Gene sequence
>lcl|BSEQ0019286|ATP-sensitive inward rectifier potassium channel 8 (KCNJ8)
ATGTTGGCCAGAAAGAGTATCATCCCGGAGGAGTATGTGCTGGCGCGCATCGCCGCAGAG
AACCTGCGCAAGCCGCGCATCCGAGACCGCCTCCCCAAAGCCCGCTTCATCGCCAAGAGC
GGGGCCTGCAACCTGGCGCATAAGAACATCCGTGAGCAAGGACGCTTTCTACAGGACATC
TTCACCACCTTGGTGGACCTGAAATGGCGCCACACGCTGGTCATCTTTACCATGTCCTTC
CTCTGCAGCTGGCTGCTCTTCGCTATCATGTGGTGGCTGGTGGCCTTTGCCCATGGGGAC
ATCTATGCTTACATGGAGAAAAGTGGAATGGAGAAAAGTGGTTTGGAGTCCACTGTGTGT
GTGACTAATGTCAGGTCTTTCACTTCTGCTTTTCTCTTCTCCATTGAAGTTCAAGTTACC
ATTGGGTTTGGAGGGAGGATGATGACAGAGGAATGCCCTTTGGCCATCACGGTTTTGATT
CTCCAGAATATTGTGGGTTTGATCATCAATGCAGTCATGTTAGGCTGCATTTTCATGAAA
ACAGCTCAGGCTCACAGAAGGGCAGAAACTTTGATTTTCAGCCGCCATGCTGTGATTGCC
GTCCGAAATGGCAAGCTGTGCTTCATGTTCCGAGTGGGTGACCTGAGGAAAAGCATGATC
ATTAGTGCCTCTGTGCGCATCCAGGTGGTCAAGAAAACAACTACACCTGAAGGGGAGGTG
GTTCCTATTCACCAACTGGACATTCCTGTTGATAACCCAATCGAGAGCAATAACATTTTT
CTGGTGGCCCCTTTGATCATCTGCCACGTGATTGACAAGCGCAGTCCCCTGTATGACATC
TCAGCAACTGACCTGGCCAACCAAGACTTGGAGGTCATAGTTATTCTGGAAGGAGTGGTT
GAAACTACTGGCATCACCACACAAGCACGAACCTCCTACATTGCTGAGGAGATCCAATGG
GGCCACCGCTTTGTGTCCATTGTGACTGAGGAAGAAGGAGTGTATTCTGTGGATTACTCC
AAATTTGGCAACACTGTTAAAGTAGCTGCTCCACGGTGCAGTGCCCGAGAGCTGGATGAG
AAACCTTCCATCCTTATTCAGACCCTCCAAAAGAGTGAACTGTCTCATCAAAATTCTCTG
AGGAAGCGCAACTCCATGAGAAGAAACAATTCCATGAGGAGGAACAATTCTATCCGAAGG
AACAATTCTTCCCTCATGGTACCAAAGGTGCAATTTATGACTCCAGAAGGAAATCAAAAC
ACATCGGAATCATGA
GenBank Gene IDD50312
GeneCard IDNot Available
GenAtlas IDKCNJ8
HGNC IDHGNC:6269
Chromosome Location12
Locus12p11.23
References
  1. Inagaki N, Inazawa J, Seino S: cDNA sequence, gene structure, and chromosomal localization of the human ATP-sensitive potassium channel, uKATP-1, gene (KCNJ8). Genomics. 1995 Nov 1;30(1):102-4. 8595887
  2. 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. 15489334
  3. Erginel-Unaltuna N, Yang WP, Blanar MA: Genomic organization and expression of KCNJ8/Kir6.1, a gene encoding a subunit of an ATP-sensitive potassium channel. Gene. 1998 Apr 28;211(1):71-8. 9573340
  4. Antzelevitch C, Yan GX: J wave syndromes. Heart Rhythm. 2010 Apr;7(4):549-58. doi: 10.1016/j.hrthm.2009.12.006. Epub 2009 Dec 11. 20153265
  5. Medeiros-Domingo A, Tan BH, Crotti L, Tester DJ, Eckhardt L, Cuoretti A, Kroboth SL, Song C, Zhou Q, Kopp D, Schwartz PJ, Makielski JC, Ackerman MJ: Gain-of-function mutation S422L in the KCNJ8-encoded cardiac K(ATP) channel Kir6.1 as a pathogenic substrate for J-wave syndromes. Heart Rhythm. 2010 Oct;7(10):1466-71. doi: 10.1016/j.hrthm.2010.06.016. Epub 2010 Jun 15. 20558321
  6. Tester DJ, Tan BH, Medeiros-Domingo A, Song C, Makielski JC, Ackerman MJ: Loss-of-function mutations in the KCNJ8-encoded Kir6.1 K(ATP) channel and sudden infant death syndrome. Circ Cardiovasc Genet. 2011 Oct;4(5):510-5. doi: 10.1161/CIRCGENETICS.111.960195. Epub 2011 Aug 11. 21836131