Gene Symbol: Kcnj1
Description: potassium voltage-gated channel subfamily J member 1
Alias: KAB-1, Kcnj, ROMK1, kir1.1, ATP-sensitive inward rectifier potassium channel 1, ATP-regulated potassium channel ROM-K, K+ channel protein, inward rectifier K(+) channel Kir1.1, potassium channel, inwardly rectifying subfamily J member 1
Species: rat
Products:     Kcnj1

Top Publications

  1. O Donnell B, Mackie T, Subramanya A, Brodsky J. Endoplasmic reticulum-associated degradation of the renal potassium channel, ROMK, leads to type II Bartter syndrome. J Biol Chem. 2017;292:12813-12827 pubmed publisher
  2. Dong K, Tang L, MacGregor G, Hebert S. Localization of the ATP/phosphatidylinositol 4,5 diphosphate-binding site to a 39-amino acid region of the carboxyl terminus of the ATP-regulated K+ channel Kir1.1. J Biol Chem. 2002;277:49366-73 pubmed
    ..7 microm). These studies suggest that the first 39 COOH-terminal amino acid residues form an ATP-PIP(2) binding domain in Kir1.1 and possibly the Kir6.x ATP-sensitive K(+) channels. ..
  3. Hu J, Qiu S, Yang F, Cao Z, Li W, Wu Y. Unique mechanism of the interaction between honey bee toxin TPNQ and rKir1.1 potassium channel explored by computational simulations: insights into the relative insensitivity of channel towards animal toxins. PLoS ONE. 2013;8:e67213 pubmed publisher
    ..1 channel towards animal toxins. ..
  4. Peters M, Ermert S, Jeck N, Derst C, Pechmann U, Weber S, et al. Classification and rescue of ROMK mutations underlying hyperprostaglandin E syndrome/antenatal Bartter syndrome. Kidney Int. 2003;64:923-32 pubmed
    ..The evaluation of different disease-causing mechanisms will be essential for establishing new and more specific therapeutic strategies for HPS/aBS patients. ..
  5. Ho K, Nichols C, Lederer W, Lytton J, Vassilev P, Kanazirska M, et al. Cloning and expression of an inwardly rectifying ATP-regulated potassium channel. Nature. 1993;362:31-8 pubmed
    ..But the presence of an H5 region, which is likely to form the ion conduction pathway, indicates that the protein may share a common origin with voltage-gated potassium channel proteins. ..
  6. Bollepalli M, Fowler P, Rapedius M, Shang L, Sansom M, Tucker S, et al. State-dependent network connectivity determines gating in a K+ channel. Structure. 2014;22:1037-46 pubmed publisher
    ..These results have important implications for our understanding of not only K+ channel gating but also the more general nature of conformational transitions that occur in other allosteric proteins. ..
  7. Frindt G, Palmer L. Effects of dietary K on cell-surface expression of renal ion channels and transporters. Am J Physiol Renal Physiol. 2010;299:F890-7 pubmed publisher
  8. Cha S, Hu M, Kurosu H, Kuro O M, Moe O, Huang C. Regulation of renal outer medullary potassium channel and renal K(+) excretion by Klotho. Mol Pharmacol. 2009;76:38-46 pubmed publisher
    ..increases the cell-membrane abundance of the renal K(+) channel renal outer medullary potassium channel 1 (ROMK1) by removing terminal sialic acids from N-glycan of the channel...
  9. Yoo D, Fang L, Mason A, Kim B, Welling P. A phosphorylation-dependent export structure in ROMK (Kir 1.1) channel overrides an endoplasmic reticulum localization signal. J Biol Chem. 2005;280:35281-9 pubmed
    The cell surface density of functional Kir1.1 (ROMK, KCNJ1) channels in the renal collecting duct is precisely regulated to maintain potassium balance. Here, we explore the mechanism by which phosphorylation of Kir1...

Scientific Experts

More Information


  1. Cao K, Tang G, Hu D, Wang R. Molecular basis of ATP-sensitive K+ channels in rat vascular smooth muscles. Biochem Biophys Res Commun. 2002;296:463-9 pubmed
    ..Our results for the first time reported the expression of four K(ATP) subunits in same vascular tissues, unmasking the diversity of native K(ATP) channels in vascular SMCs. ..
  2. Konstas A, Dabrowski M, Korbmacher C, Tucker S. Intrinsic sensitivity of Kir1.1 (ROMK) to glibenclamide in the absence of SUR2B. Implications for the identity of the renal ATP-regulated secretory K+ channel. J Biol Chem. 2002;277:21346-51 pubmed
    ..1b and is not required to confer glibenclamide sensitivity to Kir1.1b. This has important implications for the presumed structure of the renal ATP-regulated secretory K+ channel...
  3. Lopes C, Zhang H, Rohacs T, Jin T, Yang J, Logothetis D. Alterations in conserved Kir channel-PIP2 interactions underlie channelopathies. Neuron. 2002;34:933-44 pubmed
    ..We find basic residues that interact with PIP(2), two of which have been associated with Andersen's and Bartter's syndromes. We show that several naturally occurring mutants decrease channel-PIP(2) interactions, leading to disease. ..
  4. Zhou H, Tate S, Palmer L. Primary structure and functional properties of an epithelial K channel. Am J Physiol. 1994;266:C809-24 pubmed
    ..The clone appears to be a splice variant of a recently reported K channel (ROMK1) from rat renal outer medulla (Ho, K.H., C.G. Nichols, W.J. Lederer, J. Lytton, P.M. Vassilev, M.V...
  5. Lu M, Wang T, Yan Q, Yang X, Dong K, Knepper M, et al. Absence of small conductance K+ channel (SK) activity in apical membranes of thick ascending limb and cortical collecting duct in ROMK (Bartter's) knockout mice. J Biol Chem. 2002;277:37881-7 pubmed
    The ROMK (Kir1.1; Kcnj1) gene is believed to encode the apical small conductance K(+) channels (SK) of the thick ascending limb (TAL) and cortical collecting duct (CCD)...
  6. Foster D, Ho A, Rucker J, Garlid A, Chen L, Sidor A, et al. Mitochondrial ROMK channel is a molecular component of mitoK(ATP). Circ Res. 2012;111:446-54 pubmed publisher
    ..Mass spectrometric analysis was used to identify KCNJ1(ROMK) in purified bovine heart mitochondrial inner membrane and ROMK mRNA was confirmed to be present in neonatal ..
  7. Frindt G, Li H, Sackin H, Palmer L. Inhibition of ROMK channels by low extracellular K+ and oxidative stress. Am J Physiol Renal Physiol. 2013;305:F208-15 pubmed publisher
    ..1 mM. Pretreatment of the oocytes with U73122 prevented the effects of t-BHP. Under conditions of low dietary K intake, K? secretion by distal nephron segments may be suppressed by a combination of low luminal [K?]o and oxidative stress. ..
  8. Nanazashvili M, Li H, Palmer L, Walters D, Sackin H. Moving the pH gate of the Kir1.1 inward rectifier channel. Channels (Austin). 2007;1:21-8 pubmed
    ..These results support our hypothesis that hydrophobic leucines at the cytoplasmic end of the inner transmembrane helices comprise the principal pH gate of Kir1.1, a gate that can be relocated from 160-Kir1.1b to 157-Kir1.1b. ..
  9. Sackin H, Nanazashvili M, Palmer L, Krambis M, Walters D. Structural locus of the pH gate in the Kir1.1 inward rectifier channel. Biophys J. 2005;88:2597-606 pubmed
    ..1 (romk, kcnj1). kir1...
  10. Lin D, Sterling H, Lerea K, Welling P, Jin L, Giebisch G, et al. K depletion increases protein tyrosine kinase-mediated phosphorylation of ROMK. Am J Physiol Renal Physiol. 2002;283:F671-7 pubmed
    We purified His-tagged ROMK1 and carried out in vitro phosphorylation assays with (32)P-radiolabeled ATP to determine whether ROMK1 protein is a substrate for PTK...
  11. Lorenz J, Baird N, Judd L, Noonan W, Andringa A, Doetschman T, et al. Impaired renal NaCl absorption in mice lacking the ROMK potassium channel, a model for type II Bartter's syndrome. J Biol Chem. 2002;277:37871-80 pubmed
    ..These data show that the loss of ROMK in the mouse causes perturbations of electrolyte, acid-base, and fluid-volume homeostasis, reduced absorption of NaCl in the TALH, and impaired tubuloglomerular feedback. ..
  12. Lazrak A, Liu Z, Huang C. Antagonistic regulation of ROMK by long and kidney-specific WNK1 isoforms. Proc Natl Acad Sci U S A. 2006;103:1615-20 pubmed
    ..Here, we report that long WNK1 inhibited ROMK1 by stimulating its endocytosis. Inhibition of ROMK by long WNK1 was synergistic with, but not dependent on, WNK4...
  13. Felix J, Liu J, Schmalhofer W, Bailey T, Bednarek M, Kinkel S, et al. Characterization of Kir1.1 channels with the use of a radiolabeled derivative of tertiapin. Biochemistry. 2006;45:10129-39 pubmed
    ..1 channels and suggest its utility for identifying other Kir channel modulators. ..
  14. Spector D, Yang Q, Klopouh L, Deng J, Weinman E, Steplock D, et al. The ROMK potassium channel is present in mammalian urinary tract epithelia and muscle. Am J Physiol Renal Physiol. 2008;295:F1658-65 pubmed publisher
  15. Gallazzini M, Karim Z, Bichara M. Regulation of ROMK (Kir 1.1) channel expression in kidney thick ascending limb by hypertonicity: role of TonEBP and MAPK pathways. Nephron Physiol. 2006;104:126-35 pubmed
    ..These results establish that mRNA expression of ROMK is augmented in the MTAL by NaCl-induced hypertonicity through stimulation of ROMK gene transcription, and that TonEBP and the p38 MAPK and ERK pathways are involved in this effect. ..
  16. Hoagland K, Flasch A, Dahly Vernon A, dos Santos E, Knepper M, Roman R. Elevated BSC-1 and ROMK expression in Dahl salt-sensitive rat kidneys. Hypertension. 2004;43:860-5 pubmed
    ..These results suggest that overexpression of ROMK and BSC-1 in the thick ascending limb combined with a deficiency in renal formation of 20-HETE may predispose Dahl S rats fed a high-salt diet to Na+ retention and hypertension. ..
  17. Babilonia E, Wei Y, Sterling H, Kaminski P, Wolin M, Wang W. Superoxide anions are involved in mediating the effect of low K intake on c-Src expression and renal K secretion in the cortical collecting duct. J Biol Chem. 2005;280:10790-6 pubmed
    ..We conclude that O(2)(-) and related products play a role in mediating the effect of low K intake on c-Src expression and in suppressing ROMK channel activity and renal K secretion. ..
  18. Cho J, Guay Woodford L. Heterozygous mutations of the gene for Kir 1.1 (ROMK) in antenatal Bartter syndrome presenting with transient hyperkalemia, evolving to a benign course. J Korean Med Sci. 2003;18:65-8 pubmed
    ..We have identified amino acid exchanges Arg338Stop and Met357Thr in the gene exon 5 for ROMK by PCR and direct sequencing. Both mutations alter the C-terminus of the ROMK protein, and can affect channel function. ..
  19. Fila M, Brideau G, Morla L, Cheval L, Deschenes G, Doucet A. Inhibition of K+ secretion in the distal nephron in nephrotic syndrome: possible role of albuminuria. J Physiol. 2011;589:3611-21 pubmed publisher
    ..As nephrotic patients were found to display plasma potassium levels in the normal to high range, we would recommend not only a low sodium diet but also a controlled potassium diet for patients with nephrotic syndrome. ..
  20. Lin D, Sterling H, Wang Z, Babilonia E, Yang B, Dong K, et al. ROMK1 channel activity is regulated by monoubiquitination. Proc Natl Acad Sci U S A. 2005;102:4306-11 pubmed
    ..To determine the ubiquitin binding site on ROMK1, all intracellular lysine (Lys) residues of ROMK1 were individually mutated to arginine (Arg), and a two-electrode ..
  21. Dong K, Tang L, MacGregor G, Leng Q, Hebert S. Novel nucleotide-binding sites in ATP-sensitive potassium channels formed at gating interfaces. EMBO J. 2005;24:1318-29 pubmed
    ..The short N- and C-terminal segments comprising the novel intermolecular NBS are next to helices that likely move with channel opening/closing, suggesting a lock-and-key model for ligand gating. ..
  22. Boim M, Ho K, Shuck M, Bienkowski M, Block J, Slightom J, et al. ROMK inwardly rectifying ATP-sensitive K+ channel. II. Cloning and distribution of alternative forms. Am J Physiol. 1995;268:F1132-40 pubmed
    ..J. Physiol. 268 (Renal Fluid Electrolyte Physiol. 37): F1124-F1131, 1995]. Using approaches based on homology to ROMK1, we have identified two additional ROMK isoforms, ROMK2b and ROMK3...
  23. Lin D, Sterling H, Yang B, Hebert S, Giebisch G, Wang W. Protein tyrosine kinase is expressed and regulates ROMK1 location in the cortical collecting duct. Am J Physiol Renal Physiol. 2004;286:F881-92 pubmed
    ..intake regulates the expression of Src family PTK, which plays an important role in controlling the expression of ROMK1 in plasma membrane (Wei Y, Bloom P, Lin D-H, Gu RM, and Wang WH. Am J Physiol Renal Physiol 281: F206-F212, 2001)...
  24. Sackin H, Nanazashvili M, Li H, Palmer L, Walters D. A conserved arginine near the filter of Kir1.1 controls Rb/K selectivity. Channels (Austin). 2010;4:203-14 pubmed
    ..1b causing a subtle change in the selectivity filter, perhaps by disruption of an intra-subunit salt bridge (R128-E118) near the filter...
  25. Yang L, Frindt G, Palmer L. Magnesium modulates ROMK channel-mediated potassium secretion. J Am Soc Nephrol. 2010;21:2109-16 pubmed publisher
    ..These effects could play a role in the modulation of K(+) transport under conditions of K(+) and/or Mg(2+) depletion. ..
  26. Araujo M, Welch W, Zhou X, Sullivan K, Walsh S, Pasternak A, et al. Inhibition of ROMK blocks macula densa tubuloglomerular feedback yet causes renal vasoconstriction in anesthetized rats. Am J Physiol Renal Physiol. 2017;312:F1120-F1127 pubmed publisher
    ..The renal vasoconstriction was independent of volume depletion, blood pressure, TGF, or PPT. ..
  27. Chu P, Quigley R, Babich V, Huang C. Dietary potassium restriction stimulates endocytosis of ROMK channel in rat cortical collecting duct. Am J Physiol Renal Physiol. 2003;285:F1179-87 pubmed
    ..This decrease in the abundance of ROMK is likely important for maintaining K+ homeostasis during K+ deficiency. ..
  28. Palmada M, Embark H, Yun C, Böhmer C, Lang F. Molecular requirements for the regulation of the renal outer medullary K(+) channel ROMK1 by the serum- and glucocorticoid-inducible kinase SGK1. Biochem Biophys Res Commun. 2003;311:629-34 pubmed
    The serum- and glucocorticoid- inducible kinase SGK1 stimulates the renal outer medullary K(+) channel ROMK1 in the presence of the Na(+)/H(+) exchanger regulating factor NHERF2...
  29. Frindt G, Palmer L. Effects of insulin on Na and K transporters in the rat CCD. Am J Physiol Renal Physiol. 2012;302:F1227-33 pubmed publisher
    ..However, the hormone does activate both the Na/K pump and apical K(+) channels and could, under some conditions, enhance renal K(+) secretion. ..
  30. Zhang Y, Lin D, Wang Z, Jin Y, Yang B, Wang W. K restriction inhibits protein phosphatase 2B (PP2B) and suppression of PP2B decreases ROMK channel activity in the CCD. Am J Physiol Cell Physiol. 2008;294:C765-73 pubmed publisher
    ..We conclude that K restriction suppresses the expression of PP2B catalytic subunits and that inhibition of PP2B decreases ROMK channel activity through stimulation of MAPK in the CCD. ..
  31. Zhou X, Zhang Z, Shin M, Horwitz S, Levorse J, Zhu L, et al. Heterozygous disruption of renal outer medullary potassium channel in rats is associated with reduced blood pressure. Hypertension. 2013;62:288-94 pubmed publisher
    The renal outer medullary potassium channel (ROMK, KCNJ1) mediates potassium recycling and facilitates sodium reabsorption through the Na(+)/K(+)/2Cl(-) cotransporter in the loop of Henle and potassium secretion at the cortical ..
  32. Beesley A, Ortega B, White S. Splicing of a retained intron within ROMK K+ channel RNA generates a novel set of isoforms in rat kidney. Am J Physiol. 1999;276:C585-92 pubmed
    ..The deletion in this core region is predicted to generate hydrophilic proteins that are approximately one-third of the size of native ROMK and lack membrane-spanning domains. ..
  33. Lin D, Kamsteeg E, Zhang Y, Jin Y, Sterling H, Yue P, et al. Expression of tetraspan protein CD63 activates protein-tyrosine kinase (PTK) and enhances the PTK-induced inhibition of ROMK channels. J Biol Chem. 2008;283:7674-81 pubmed publisher
    In the present study, we tested the role of CD63 in regulating ROMK1 channels by protein-tyrosine kinase (PTK)...
  34. Lin D, Yue P, Pan C, Sun P, Zhang X, Han Z, et al. POSH stimulates the ubiquitination and the clathrin-independent endocytosis of ROMK1 channels. J Biol Chem. 2009;284:29614-24 pubmed publisher
    ..Moreover, immunoprecipitation of lysates of HEK293T cells transfected with ROMK1 or with constructs encoding the ROMK-N terminus or ROMK1-C-Terminus demonstrated that POSH binds to ROMK1 on its N ..
  35. Fallen K, Banerjee S, Sheehan J, Addison D, Lewis L, Meiler J, et al. The Kir channel immunoglobulin domain is essential for Kir1.1 (ROMK) thermodynamic stability, trafficking and gating. Channels (Austin). 2009;3:57-68 pubmed
    ..Our study sheds new light on the pathogenesis of ABS and establishes the IgLD as an essential structure within the Kir channel family. ..
  36. Ruete M, Carrizo L, Bocanegra M, Valles P. Altered renal expression of Na(+) transporters and ROMK in protein-deprived rats. Nephron Physiol. 2009;111:p17-29 pubmed publisher
    ..A role of aldosterone may be suggested. ..
  37. Smoot B, Kober K, Paul S, Levine J, Abrams G, Mastick J, et al. Potassium Channel Candidate Genes Predict the Development of Secondary Lymphedema Following Breast Cancer Surgery. Nurs Res. 2017;66:85-94 pubmed publisher
    ..These preliminary findings suggest that K channel genes play a role in the development of secondary LE. ..