KCNJ10 determines the expression of the apical Na-Cl cotransporter (NCC) in the early distal convoluted tubule (DCT1)

Proceedings of the National Academy of Sciences of the United States of America

Volumn 111, Issue 32, 2014, Pages 11864-11869

  Zhang, Chengbiao ^a,b   Wang, Lijun ^b   Zhang, Junhui ^c   Su, Xiao Tong ^b   Lin, Dao Hong ^b   Scholl, Ute I ^c   Giebisch, Gerhard ^d   Lifton, Richard P ^c   Wang, Wen Hui ^b  

^a XUZHOU MEDICAL UNIVERSITY   (China)

^b NEW YORK MEDICAL COLLEGE   (United States)

^c HOWARD HUGHES MEDICAL INSTITUTE   (United States)

^d YALE UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Kir.5.1; SeSAME EAST syndrome; SPAK; WNK

Indexed keywords

INWARDLY RECTIFYING POTASSIUM CHANNEL; KCNJ10 PROTEIN; KCNJ16 PROTEIN; POTASSIUM CHANNEL; PROLINE; SODIUM CHLORIDE COTRANSPORTER; UNCLASSIFIED DRUG;

ADULT; ANIMAL EXPERIMENT; ARTICLE; BASOLATERAL MEMBRANE; CELL MEMBRANE POTENTIAL; CONTROLLED STUDY; FEMALE; IMMUNOHISTOCHEMISTRY; KIDNEY DISTAL TUBULE; MOUSE; NONHUMAN; PATCH CLAMP TECHNIQUE; POTASSIUM CONDUCTANCE; POTASSIUM CURRENT; PRIORITY JOURNAL; PROTEIN EXPRESSION; WESTERN BLOTTING;

KIR.5.1; SESAME/EAST SYNDROME; SPAK; WNK;

ANIMALS; IMMUNOHISTOCHEMISTRY; KIDNEY TUBULES, DISTAL; MEMBRANE POTENTIALS; MICE; MICE, KNOCKOUT; MODELS, BIOLOGICAL; PATCH-CLAMP TECHNIQUES; POTASSIUM CHANNELS, INWARDLY RECTIFYING; SOLUTE CARRIER FAMILY 12, MEMBER 3;

EID: 84905980010     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1411705111     Document Type: Article

References (44)

1. Monroy A, Plata C, Hebert SC, Gamba G (2000) Characterization of the thiazidesensitive Na(+)-Cl(-) cotransporter: A new model for ions and diuretics interaction. Am J Physiol Renal Physiol 279(1):F161-F169
2. Ellison DH, Valazquez H, Wright FS (1987) Thiazide-sensitive sodium chloride cotransport in early distal tubule. Am J Physiol Renal Physiol 253(3 Pt 2):F546-F554
3. Simon DB, et al. (1996) Gitelman's variant of Bartter's syndrome, inherited hypokalaemic alkalosis, is caused by mutations in the thiazide-sensitive Na-Cl cotransporter. Nat Genet 12(1):24-30
4. Bachmann S, et al. (1995) Expression of the thiazide-sensitive Na-Cl cotransporter by rabbit distal convoluted tubule cells. J Clin Invest 96(5):2510-2514
5. Rubera I, et al. (2003) Collecting duct-specific gene inactivation of alphaENaC in the mouse kidney does not impair sodium and potassium balance. J Clin Invest 112(4): 554-565
6. Wade JB, et al. (2011) Differential regulation of ROMK (Kir1.1) in distal nephron segments by dietary potassium. Am J Physiol Renal Physiol 300(6):F1385-F1393
7. Lourdel S, Paulais M, Marvao P, Nissant A, Teulon J (2003) A chloride channel at the basolateral membrane of the distal-convoluted tubule: A candidate ClC-K channel. J Gen Physiol 121(4):287-300
8. Liapis H, Nag M, Kaji DM (1998) K-Cl cotransporter expression in the human kidney. Am J Physiol 275(6 Pt 1):C1432-C1437
9. Hebert SC, Desir G, Giebisch G, Wang W (2005) Molecular diversity and regulation of renal potassium channels. Physiol Rev 85(1):319-371
10. Wang W, Hebert SC, Giebisch G (1997) Renal K+ channels: Structure and function. Annu Rev Physiol 59:413-436
11. Lourdel S, et al. (2002) An inward rectifier K(+) channel at the basolateral membrane of the mouse distal convoluted tubule: Similarities with Kir4-Kir5.1 heteromeric channels. J Physiol 538(Pt 2):391-404
12. Williams DM, et al. (2010) Molecular basis of decreased Kir4.1 function in SeSAME/EAST syndrome. J Am Soc Nephrol 21(12):2117-2129
13. Lachheb S, et al. (2008) Kir4.1/Kir5.1 channel forms the major K+ channel in the basolateral membrane of mouse renal collecting duct principal cells. Am J Physiol Renal Physiol 294(6):F1398-F1407
14. Cha SK, et al. (2011) Calcium-sensing receptor decreases cell surface expression of the inwardly rectifying K+ channel Kir4.1. J Biol Chem 286(3):1828-1835
15. Zhang C, et al. (2013) Src family protein tyrosine kinase regulates the basolateral K channel in the distal convoluted tubule (DCT) by phosphorylation of KCNJ10 protein. J Biol Chem 288(36):26135-26146
16. Bockenhauer D, et al. (2009) Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations. N Engl J Med 360(19):1960-1970
17. Reichold M, et al. (2010) KCNJ10 gene mutations causing EAST syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) disrupt channel function. Proc Natl Acad Sci USA 107(32):14490-14495
18. Scholl UI, et al. (2009) Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SeSAME syndrome) caused by mutations in KCNJ10. Proc Natl Acad Sci USA 106(14):5842-5847
19. Paulais M, et al. (2011) Renal phenotype in mice lacking the Kir5.1 (Kcnj16) K+ channel subunit contrasts with that observed in SeSAME/EAST syndrome. Proc Natl Acad Sci USA 108(25):10361-10366
20. Kofuji P, et al. (2000) Genetic inactivation of an inwardly rectifying potassium channel (Kir4.1 subunit) in mice: Phenotypic impact in retina. J Neurosci 20(15):5733-5740
21. Neusch C, Rozengurt N, Jacobs RE, Lester HA, Kofuji P (2001) Kir4.1 potassium channel subunit is crucial for oligodendrocyte development and in vivo myelination. J Neurosci 21(15):5429-5438
22. Bandulik S, et al. (2011) The salt-wasting phenotype of EAST syndrome, a disease with multifaceted symptoms linked to the KCNJ10 K+ channel. Pflugers Arch 461(4): 423-435
23. Belge H, et al. (2007) Renal expression of parvalbumin is critical for NaCl handling and response to diuretics. Proc Natl Acad Sci USA 104(37):14849-14854
24. Pessia M, Tucker SJ, Lee K, Bond CT, Adelman JP (1996) Subunit positional effects revealed by novel heteromeric inwardly rectifying K+ channels. EMBO J 15(12): 2980-2987
25. Piala AT, et al. (2014) Chloride sensing by WNK1 involves inhibition of autophosphorylation. Sci Signal 7:ra41
26. Ponce-Coria J, et al. (2008) Regulation of NKCC2 by a chloride-sensing mechanism involving the WNK3 and SPAK kinases. Proc Natl Acad Sci USA 105(24):8458-8463
27. San-Cristobal P, et al. (2009) Angiotensin II signaling increases activity of the renal Na-Cl cotransporter through a WNK4-SPAK-dependent pathway. Proc Natl Acad Sci USA 106(11):4384-4389
28. Piechotta K, Lu J, Delpire E (2002) Cation chloride cotransporters interact with the stress-related kinases Ste20-related proline-alanine-rich kinase (SPAK) and oxidative stress response 1 (OSR1). J Biol Chem 277(52):50812-50819
29. McCormick JA, et al. (2011) A SPAK isoform switch modulates renal salt transport and blood pressure. Cell Metab 14(3):352-364
30. Schultz SG (1981) Homocellular regulatory mechanisms in sodium-transporting epithelia: Avoidance of extinction by "flush-through. " Am J Physiol 241(6):F579-F590
31. Zheng W, et al. (2007) Cellular distribution of the potassium channel KCNQ1 in normal mouse kidney. Am J Physiol Renal Physiol 292(1):F456-F466
32. Suzuki Y, et al. (2003) Expression of the K+ channel Kir7.1 in the developing rat kidney: Role in K+ excretion. Kidney Int 63(3):969-975
33. Ookata K, et al. (2000) Localization of inward rectifier potassium channel Kir7.1 in the basolateral membrane of distal nephron and collecting duct. J Am Soc Nephrol 11(11):1987-1994
34. Lalioti MD, et al. (2006) Wnk4 controls blood pressure and potassium homeostasis via regulation of mass and activity of the distal convoluted tubule. Nat Genet 38(10): 1124-1132
35. Yang CL, Angell J, Mitchell R, Ellison DH (2003) WNK kinases regulate thiazide-sensitive Na-Cl cotransport. J Clin Invest 111(7):1039-1045
36. Rozansky DJ, et al. (2009) Aldosterone mediates activation of the thiazide-sensitive Na-Cl cotransporter through an SGK1 and WNK4 signaling pathway. J Clin Invest 119(9):2601-2612
37. Castañeda-Bueno M, et al. (2012) Activation of the renal Na+:Cl-cotransporter by angiotensin II is a WNK4-dependent process. Proc Natl Acad Sci USA 109(20): 7929-7934
38. Shibata S, Zhang J, Puthumana J, Stone KL, Lifton RP (2013) Kelch-like 3 and Cullin 3 regulate electrolyte homeostasis via ubiquitination and degradation of WNK4. Proc Natl Acad Sci USA 110(19):7838-7843
39. Boyden LM, et al. (2012) Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities. Nature 482(7383):98-102
40. Rosenbaek LL, Kortenoeven MLA, Aroankins TS, Fenton RA (2014) Phosphorylation decreases ubiquitylation of the thiazide-sensitive cotransporter NCC and subsequent clathrin-mediated endocytosis. J Biol Chem 289(19):13347-13361
41. Yang SS, et al. (2010) SPAK-knockout mice manifest Gitelman syndrome and impaired vasoconstriction. J Am Soc Nephrol 21(11):1868-1877
42. Loffing J, et al. (2004) Altered renal distal tubule structure and renal Na(+) and Ca(2+) handling in a mouse model for Gitelman's syndrome. J Am Soc Nephrol 15(9): 2276-2288
43. Houillier P (2014) Mechanisms and regulation of renal magnesium transport. Annu Rev Physiol 76(1):411-430
44. Ko B, et al. (2012) A new model of the distal convoluted tubule. Am J Physiol Renal Physiol 303(5):F700-F710.