Gene Symbol: KCNJ16
Description: potassium voltage-gated channel subfamily J member 16
Alias: BIR9, KIR5.1, inward rectifier potassium channel 16, inward rectifier K(+) channel Kir5.1, inward rectifier K+ channel KIR5.1, potassium channel, inwardly rectifying subfamily J member 16, potassium inwardly-rectifying channel, subfamily J, member 16
Species: human
Products:     KCNJ16

Top Publications

  1. Derst C, Karschin C, Wischmeyer E, Hirsch J, Preisig Müller R, Rajan S, et al. Genetic and functional linkage of Kir5.1 and Kir2.1 channel subunits. FEBS Lett. 2001;491:305-11 pubmed
    ..The hKir5.1 gene KCNJ16 is assigned to chromosomal region 17q23.1-24.2, and is separated by only 34 kb from the hKir2.1 gene (KCNJ2)...
  2. Pereyra S, Sosa C, Bertoni B, Sapiro R. Transcriptomic analysis of fetal membranes reveals pathways involved in preterm birth. BMC Med Genomics. 2019;12:53 pubmed publisher
    ..genes are involved in the nervous system, morphogenesis (WNT1, DLX5, PAPPA2) and ion channel complexes (KCNJ16, KCNB1), making them good candidates as biomarkers of PTB...
  3. Xiao Y, Meng X, Zhang H, Guo X, Gu R. [The function and regulation of basolateral Kir4.1 and Kir4.1/Kir5.1 in renal tubules]. Sheng Li Xue Bao. 2018;70:600-606 pubmed
    ..Here we review the progress of renal tubule basolateral Kir, and mainly discuss the function and regulation of Kir4.1 and Kir4.1/Kir5.1. ..
  4. Wu P, Gao Z, Su X, Wang M, Wang W, Lin D. Kir4.1/Kir5.1 Activity Is Essential for Dietary Sodium Intake-Induced Modulation of Na-Cl Cotransporter. J Am Soc Nephrol. 2019;30:216-227 pubmed publisher
    ..Stimulation of Kir4.1/Kir5.1 by low intake of dietary sodium is essential for NCC upregulation, and inhibition of Kir4.1/Kir5.1 induced by high sodium intake is a key step for downregulation of NCC. ..
  5. Sala Rabanal M, Kucheryavykh L, Skatchkov S, Eaton M, Nichols C. Molecular mechanisms of EAST/SeSAME syndrome mutations in Kir4.1 (KCNJ10). J Biol Chem. 2010;285:36040-8 pubmed publisher
    ..These results provide an explanation for the molecular defects that underlie the EAST/SeSAME syndrome. ..
  6. Li J, Tang S, Zheng J, Tian C, Liu L. Removable Backbone Modification Method for the Chemical Synthesis of Membrane Proteins. Acc Chem Res. 2017;50:1143-1153 pubmed publisher
    ..The RBM method enables the practical engineering of small- to medium-sized MPs or membrane protein domains to address fundamental questions in the biochemical, biophysical, and pharmaceutical sciences. ..
  7. Wang L, Zhang C, Su X, Lin D, Wang W. Caveolin-1 Deficiency Inhibits the Basolateral K+ Channels in the Distal Convoluted Tubule and Impairs Renal K+ and Mg2+ Transport. J Am Soc Nephrol. 2015;26:2678-90 pubmed publisher
    ..Furthermore, the decrease in Kcnj10 and Na(+)/Cl(-) cotransporter expression induced by cav-1 deficiency may underlie the compromised renal transport of Mg(2+), Ca(2+), and K(+). ..
  8. Thomson S, Hansen A, Sanguinetti M. Identification of the Intracellular Na+ Sensor in Slo2.1 Potassium Channels. J Biol Chem. 2015;290:14528-35 pubmed publisher
    ..1 currents by 3 mM niflumic acid was 14-fold greater than activation achieved by increasing [NaCl]i from 3 to 100 mM. Thus, relative to fenamates, intracellular Na(+) is a poor activator of Slo2.1. ..
  9. Ramos H, da Silva M, Carré A, Silva J, Paninka R, Oliveira T, et al. Molecular insights into the possible role of Kir4.1 and Kir5.1 in thyroid hormone biosynthesis. Horm Res Paediatr. 2015;83:141-7 pubmed publisher
    ..The role of Kir in thyroid morphogenesis and differentiation might be understood in future studies. We speculate that thyroglobulin trafficking might be modulated by Kir4.1/5.1. ..

More Information


  1. D Adamo M, Shang L, Imbrici P, Brown S, Pessia M, Tucker S. Genetic inactivation of Kcnj16 identifies Kir5.1 as an important determinant of neuronal PCO2/pH sensitivity. J Biol Chem. 2011;286:192-8 pubmed publisher
    ..To examine this, we generated mutant mice lacking the Kir5.1 (Kcnj16) gene...
  2. Trapp S, Tucker S, Gourine A. Respiratory responses to hypercapnia and hypoxia in mice with genetic ablation of Kir5.1 (Kcnj16). Exp Physiol. 2011;96:451-9 pubmed publisher
    ..These results therefore suggest that despite their intrinsic pH sensitivity, Kir4.1-Kir5.1 channels are dispensable for functional central and peripheral respiratory chemosensitivity. ..
  3. Lee Y, Shin D, Song K. Dominance effects of ion transport and ion transport regulator genes on the final weight and backfat thickness of Landrace pigs by dominance deviation analysis. Genes Genomics. 2018;40:1331-1338 pubmed publisher
    ..effects in final weight and overdominant effects in backfat thickness were ion transport with the SLC8A3, KCNJ16, P2RX7 and TRPC3 genes...
  4. Inanobe A, Itamochi H, Kurachi Y. Kir Channel Blockages by Proflavine Derivatives via Multiple Modes of Interaction. Mol Pharmacol. 2018;93:592-600 pubmed publisher
    ..In conclusion, preferential blockage of Kir-channel family members by proflavine derivatives is based on multiple modes of action. This raises the possibility of designing subunit-specific inhibitors. ..
  5. Brasko C, Hawkins V, De La Rocha I, Butt A. Expression of Kir4.1 and Kir5.1 inwardly rectifying potassium channels in oligodendrocytes, the myelinating cells of the CNS. Brain Struct Funct. 2017;222:41-59 pubmed publisher
    ..We propose Kir4.1/Kir5.1 channels have equivalent functions in oligodendrocytes, maintaining myelin integrity in the face of large ionic shifts associated with action potential propagation along myelinated axons. ..
  6. Méndez González M, Kucheryavykh Y, Zayas Santiago A, Vélez Carrasco W, Maldonado Martínez G, Cubano L, et al. Novel KCNJ10 Gene Variations Compromise Function of Inwardly Rectifying Potassium Channel 4.1. J Biol Chem. 2016;291:7716-26 pubmed publisher
    ..1 channels achieved a higher block than WT, suggesting a more stable interaction of SPM in the deep pore cavity. Overall, our data suggest that G83V, L166Q, and Q212R residues play a pivotal role in controlling Kir4.1 channel function. ..
  7. Kompatscher A, de Baaij J, Aboudehen K, Hoefnagels A, Igarashi P, Bindels R, et al. Loss of transcriptional activation of the potassium channel Kir5.1 by HNF1β drives autosomal dominant tubulointerstitial kidney disease. Kidney Int. 2017;92:1145-1156 pubmed publisher
    ..A highly specific and conserved HNF1β site was identified in the promoter of Kcnj16 that encodes the potassium channel Kir5.1. Luciferase-promoter assays showed a 2...
  8. Puissant M, Mouradian G, Liu P, Hodges M. Identifying Candidate Genes that Underlie Cellular pH Sensitivity in Serotonin Neurons Using Transcriptomics: A Potential Role for Kir5.1 Channels. Front Cell Neurosci. 2017;11:34 pubmed publisher
    ..1), kcnj16 (Kir5.1), kcnk1 (TWIK-1), kcnk3 (TASK-1) and kcnk9 (TASK-3)...
  9. Perez Samartin A, Garay E, Moctezuma J, Cisneros Mejorado A, Sánchez Gómez M, Martel Gallegos G, et al. Inwardly Rectifying K+ Currents in Cultured Oligodendrocytes from Rat Optic Nerve are Insensitive to pH. Neurochem Res. 2017;42:2443-2455 pubmed publisher
    ..It is therefore concluded that Kir channel H+-sensitivity in OLs is modulated through an extrinsic mechanism, probably by association with a modulatory component or by posttranslational modifications. ..
  10. Pan C, Chu H, Lai Y, Sun Y, Du Z, Liu Y, et al. Downregulation of inwardly rectifying potassium channel 5.1 expression in C57BL/6J cochlear lateral wall. J Huazhong Univ Sci Technolog Med Sci. 2016;36:406-409 pubmed publisher
    ..It was suggested that the age-related decreased expression of Kir5.1 in the lateral wall of C57BL/6J mice was associated with hearing loss. Our results indicated that Kir5.1 may play an important role in the pathogenesis of AHL. ..
  11. Welling P. Roles and Regulation of Renal K Channels. Annu Rev Physiol. 2016;78:415-35 pubmed publisher
    ..special function of five different potassium channels in the distal nephron, encoded by the genes KCNJ1, KCNJ10, KCNJ16, KCNMA1, and KCNN3...
  12. Fatemifar G, Hoggart C, Paternoster L, Kemp J, Prokopenko I, Horikoshi M, et al. Genome-wide association study of primary tooth eruption identifies pleiotropic loci associated with height and craniofacial distances. Hum Mol Genet. 2013;22:3807-17 pubmed publisher
    ..Our results suggest that the genome-wide association approach is a powerful strategy for detecting variants involved in tooth eruption, and potentially craniofacial growth and more generally organ development. ..
  13. Castori M, Bottillo I, Morlino S, Barone C, Cascone P, Grammatico P, et al. Variability in a three-generation family with Pierre Robin sequence, acampomelic campomelic dysplasia, and intellectual disability due to a novel ∼1 Mb deletion upstream of SOX9, and including KCNJ2 and KCNJ16. Birth Defects Res A Clin Mol Teratol. 2016;106:61-8 pubmed publisher
    ..We report a three-generation pedigree with a novel ∼1 Mb deletion upstream of SOX9 and including KCNJ2 and KCNJ16, and ascertained for dominant transmission of PRS...
  14. Jin X, Yu L, Wu Y, Zhang S, Shi Z, Chen X, et al. S-Glutathionylation underscores the modulation of the heteromeric Kir4.1-Kir5.1 channel in oxidative stress. J Physiol. 2012;590:5335-48 pubmed publisher
    ..The finding that the heteromeric Kir4.1-Kir5.1 channel but not the homomeric Kir4.1 is subject to the S-glutathionylation thus suggests a novel Kir4.1-Kir5.1 channel modulation mechanism that is likely to occur in oxidative stress. ..
  15. Liu G, Wu K, Sheng Y. Elucidation of the molecular mechanisms of anaplastic thyroid carcinoma by integrated miRNA and mRNA analysis. Oncol Rep. 2016;36:3005-3013 pubmed publisher
    ..Gene expression levels of three randomly selected DEGs, VCAN, COL5A1 and KCNJ16, were examined using RT-PCR in 10 ATC samples...
  16. Bouhouche A, Benomar A, Errguig L, Lachhab L, Bouslam N, Aasfara J, et al. An autosomal recessive leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa maps to chromosome 17q24.2-25.3. BMC Med Genet. 2012;13:18 pubmed publisher
    ..90. Sequencing of seven candidate genes in this locus, ATP5H, FDXR, SLC25A19, MCT8, CYGB, KCNJ16 and GRIN2C, identified three missense mutations in the FDXR gene which were also found in a homozygous state in ..
  17. Fowler C, Aryal P, Suen K, Slesinger P. Evidence for association of GABA(B) receptors with Kir3 channels and regulators of G protein signalling (RGS4) proteins. J Physiol. 2007;580:51-65 pubmed
    ..2a and RGS4 nor between Kir3.2a and Galphao. These data are discussed in terms of a model in which GABA(B) receptors, G proteins, RGS4 proteins and Kir3 channels are closely associated in a signalling complex. ..
  18. Casamassima M, D Adamo M, Pessia M, Tucker S. Identification of a heteromeric interaction that influences the rectification, gating, and pH sensitivity of Kir4.1/Kir5.1 potassium channels. J Biol Chem. 2003;278:43533-40 pubmed
    ..The highly conserved nature of this structural association between Kir subunits also has implications for understanding the general mechanisms of Kir channel gating and their regulation by intracellular pH. ..
  19. Tanemoto M, Fujita A, Higashi K, Kurachi Y. PSD-95 mediates formation of a functional homomeric Kir5.1 channel in the brain. Neuron. 2002;34:387-97 pubmed
    ..1/PSD-95 were suppressed promptly and reversibly by PKA activation. Because the Kir5.1/PSD-95 complex was detected in the brain, this functional brain K+ channel is potentially a novel physiological target of PKA-mediated signaling. ..
  20. Hasan S, Balobaid A, Grottesi A, Dabbagh O, Cenciarini M, Rawashdeh R, et al. Lethal digenic mutations in the K+ channels Kir4.1 (KCNJ10) and SLACK (KCNT1) associated with severe-disabling seizures and neurodevelopmental delay. J Neurophysiol. 2017;118:2402-2411 pubmed publisher
    ..This study provides new insights into the phenotypic spectrum and to the genotype-phenotype correlations associated with EAST/SeSAME and MMFSI. ..
  21. Pearson W, Dourado M, Schreiber M, Salkoff L, Nichols C. Expression of a functional Kir4 family inward rectifier K+ channel from a gene cloned from mouse liver. J Physiol. 1999;514 ( Pt 3):639-53 pubmed
    ..2 channel gene product forms functional channels in Xenopus oocytes, that these Kir channels display novel properties, and that Kir4.2 subunits may be responsible for physiological modulation of functional Kir channels. ..
  22. Zhang C, Wang L, Thomas S, Wang K, Lin D, Rinehart J, et al. Src family protein tyrosine kinase regulates the basolateral K channel in the distal convoluted tubule (DCT) by phosphorylation of KCNJ10 protein. J Biol Chem. 2013;288:26135-46 pubmed publisher
    ..a 19-pS K channel in KCNJ10-transfected HEK293T cells and a 40-pS K channel in the cells transfected with KCNJ10+KCNJ16 (Kir.5.1) that form a heterotetramer in the basolateral membrane of the DCT...
  23. Cheung C, Lau K, Ho A, Lee K, Tiu S, Lau E, et al. Genome-wide association study identifies a susceptibility locus for thyrotoxic periodic paralysis at 17q24.3. Nat Genet. 2012;44:1026-9 pubmed publisher
    ..0001). Our study has identified a susceptibility locus associated with TPP and provides insight into the causes of TPP...
  24. Benko S, Fantes J, Amiel J, Kleinjan D, Thomas S, Ramsay J, et al. Highly conserved non-coding elements on either side of SOX9 associated with Pierre Robin sequence. Nat Genet. 2009;41:359-64 pubmed publisher
    ..Some cases of PRS may thus result from developmental misexpression of SOX9 due to disruption of very-long-range cis-regulatory elements...
  25. Konstas A, Korbmacher C, Tucker S. Identification of domains that control the heteromeric assembly of Kir5.1/Kir4.0 potassium channels. Am J Physiol Cell Physiol. 2003;284:C910-7 pubmed
  26. Pessia M, Imbrici P, D Adamo M, Salvatore L, Tucker S. Differential pH sensitivity of Kir4.1 and Kir4.2 potassium channels and their modulation by heteropolymerisation with Kir5.1. J Physiol. 2001;532:359-67 pubmed
    ..g. pancreas) and that these novel channels are likely to be regulated by changes in intracellular pH. In addition, the extreme pH sensitivity of Kir4.2 has implications for the role of this subunit as a homotetrameric channel. ..
  27. Juang J, Lu T, Lai L, Ho C, Liu Y, Tsai C, et al. Disease-targeted sequencing of ion channel genes identifies de novo mutations in patients with non-familial Brugada syndrome. Sci Rep. 2014;4:6733 pubmed publisher
    ..Five de novo mutations were identified in four genes (SCNN1A, KCNJ16, KCNB2, and KCNT1) in three BrS patients (20%). Two of the three patients presented SCD and one had syncope...
  28. Jiang S, Zhu L, Yang J, Hu L, Gu J, Xing X, et al. Integrated expression profiling of potassium channels identifys KCNN4 as a prognostic biomarker of pancreatic cancer. Biochem Biophys Res Commun. 2017;494:113-119 pubmed publisher
    ..PDAC and matching normal pancreatic tissue samples, four differentially expressed K+ channels (KCNJ5, KCNJ16, KCNN4 and KCNK1) were identified in PDAC...
  29. Zheng J, Yu M, Qi Y, Tang S, Shen F, Wang Z, et al. Expedient total synthesis of small to medium-sized membrane proteins via Fmoc chemistry. J Am Chem Soc. 2014;136:3695-704 pubmed publisher
    ..1. Functional characterizations of these chemically synthesized membrane proteins indicate that they provide useful and otherwise-difficult-to-access materials for biochemistry and biophysics studies. ..
  30. Parrock S, Hussain S, Issler N, Differ A, Lench N, Guarino S, et al. KCNJ10 mutations display differential sensitivity to heteromerisation with KCNJ16. Nephron Physiol. 2013;123:7-14 pubmed publisher
    ..A167V only, which was not compatible with causing disease. However, co-expression with KCNJ16 abolished function in these heteromeric channels almost completely...
  31. Brochiero E, Wallendorf B, Gagnon D, Laprade R, Lapointe J. Cloning of rabbit Kir6.1, SUR2A, and SUR2B: possible candidates for a renal K(ATP) channel. Am J Physiol Renal Physiol. 2002;282:F289-300 pubmed
    ..x with or without the SUR subunit were significantly inhibited by taurine. This study suggests that the taurine-sensitive K(ATP) channel of rabbit proximal tubules is formed by a combination of Kir6.1 plus SUR2A and/or SUR2B...
  32. Liu Y, McKenna E, Figueroa D, Blevins R, Austin C, Bennett P, et al. The human inward rectifier K(+) channel subunit kir5.1 (KCNJ16) maps to chromosome 17q25 and is expressed in kidney and pancreas. Cytogenet Cell Genet. 2000;90:60-3 pubmed
    A novel human Kir5.1 (inward rectifier K+ channel subunit, gene name KCNJ16) was identified through database searches. This human KCNJ16 was mapped to chromosome 17q25...
  33. Kubo Y, Adelman J, Clapham D, Jan L, Karschin A, Kurachi Y, et al. International Union of Pharmacology. LIV. Nomenclature and molecular relationships of inwardly rectifying potassium channels. Pharmacol Rev. 2005;57:509-26 pubmed