Disease-causing mutations in KLHL3 impair its effect on WNK4 degradation

FEBS Letters

Volumn 587, Issue 12, 2013, Pages 1717-1722

  Wu, Guojin ^a   Peng, Ji Bin ^a  

^a University of Alabama at Birmingham   (United States)

Author keywords

Cullin 3 with no lysine (K) kinase 4; kelch like 3; Ubiquitin E3 ligase; Ubiquitination

Indexed keywords

ADAPTOR PROTEIN; CULLIN; CULLIN 3; PHOSPHOTRANSFERASE; PROTEIN KINASE WNK4; PROTEIN KLHL3; PROTEIN OSR1; SODIUM CHLORIDE COTRANSPORTER; UBIQUITIN PROTEIN LIGASE E3; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME ANALYSIS; ENZYME DEGRADATION; ENZYME INDUCTION; ENZYME INHIBITION; ENZYME REGULATION; ENZYME SUBSTRATE COMPLEX; GENE MUTATION; HUMAN; HYPERTENSION; MUTATIONAL ANALYSIS; NONHUMAN; NUCLEOTIDE SEQUENCE; OOCYTE; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN FUNCTION; PROTEIN TARGETING; PSEUDOHYPOALDOSTERONISM; UBIQUITINATION; XENOPUS;

ANIMALS; CARRIER PROTEINS; CULLIN PROTEINS; HUMANS; MICE; MUTATION; PROTEIN STABILITY; PROTEIN-SERINE-THREONINE KINASES; PROTEOLYSIS; PSEUDOHYPOALDOSTERONISM; RECEPTORS, DRUG; SYMPORTERS; UBIQUITIN-PROTEIN LIGASES; UBIQUITINATION;

EID: 84878774970     PISSN: 00145793     EISSN: 18733468     Source Type: Journal    
DOI: 10.1016/j.febslet.2013.04.032     Document Type: Article

References (30)

1. W.K. Paver, and G.J. Pauline Hypertension and hyperpotassaemia without renal disease in a young male Med. J. Aust. 2 1964 305 306
2. R.D. Gordon, R.A. Geddes, C.G. Pawsey, and M.W. O'Halloran Hypertension and severe hyperkalaemia associated with suppression of renin and aldosterone and completely reversed by dietary sodium restriction Australas. Ann. Med. 19 1970 287 294
3. F.H. Wilson, S. Disse-Nicodeme, and K.A. Choate Human hypertension caused by mutations in WNK kinases Science 293 2001 1107 1112
4. T. Na, G. Wu, and J.B. Peng Disease-causing mutations in the acidic motif of WNK4 impair the sensitivity of WNK4 kinase to calcium ions Biochem. Biophys. Res. Commun. 419 2012 293 298
5. T. Na, G. Wu, W. Zhang, W.J. Dong, and J.B. Peng Disease-causing R1185C mutation of WNK4 disrupts a regulatory mechanism involving calmodulin binding and SGK1 phosphorylation sites Am. J. Physiol. Renal Physiol. 304 2012 F8 F18
6. + homeostasis Proc. Natl. Acad. Sci. USA 104 2007 4025 4029
7. D.J. Rozansky, T. Cornwall, and A.R. Subramanya Aldosterone mediates activation of the thiazide-sensitive Na-Cl cotransporter through an SGK1 and WNK4 signaling pathway J. Clin. Invest. 119 2009 2601 2612
8. M.D. Lalioti, J. Zhang, and H.M. Volkman Wnk4 controls blood pressure and potassium homeostasis via regulation of mass and activity of the distal convoluted tubule Nat. Genet. 38 2006 1124 1132
9. D561A/+ knockin mouse model Cell Metab. 5 2007 331 344
10. - cotransporter by angiotensin II is a WNK4-dependent process Proc. Natl. Acad. Sci. USA 109 2012 7929 7934
11. C.L. Yang, J. Angell, R. Mitchell, and D.H. Ellison WNK kinases regulate thiazide-sensitive Na-Cl cotransport J. Clin. Invest. 111 2003 1039 1045
12. F.H. Wilson, K.T. Kahle, and E. Sabath Molecular pathogenesis of inherited hypertension with hyperkalemia: the Na-Cl cotransporter is inhibited by wild-type but not mutant WNK4 Proc. Natl. Acad. Sci. USA 100 2003 680 684
13. B. Zhou, J. Zhuang, and D. Gu WNK4 enhances the degradation of NCC through a sortilin-mediated lysosomal pathway J. Am. Soc. Nephrol. 21 2010 82 92
14. A.R. Subramanya, J. Liu, D.H. Ellison, J.B. Wade, and P.A. Welling WNK4 diverts the thiazide-sensitive NaCl cotransporter to the lysosome and stimulates AP-3 interaction J. Biol. Chem. 284 2009 18471 18480
15. A.C. Vitari, M. Deak, N.A. Morrice, and D.R. Alessi The WNK1 and WNK4 protein kinases that are mutated in Gordon's hypertension syndrome phosphorylate and activate SPAK and OSR1 protein kinases Biochem. J. 391 2005 17 24
16. - cotransporter by the WNK-regulated kinases SPAK and OSR1 J. Cell Sci. 121 2008 675 684
17. P. San-Cristobal, D. Pacheco-Alvarez, and C. Richardson Angiotensin II signaling increases activity of the renal Na-Cl cotransporter through a WNK4-SPAK-dependent pathway Proc. Natl. Acad. Sci. USA 106 2009 4384 4389
18. L.M. Boyden, M. Choi, and K.A. Choate Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities Nature 482 2012 98 102
19. H. Louis-Dit-Picard, J. Barc, and D. Trujillano KLHL3 mutations cause familial hyperkalemic hypertension by impairing ion transport in the distal nephron Nat. Genet. 44 2012 456 463
20. M. Furukawa, Y.J. He, C. Borchers, and Y. Xiong Targeting of protein ubiquitination by BTB-Cullin 3-Roc1 ubiquitin ligases Nat. Cell Biol. 5 2003 1001 1007
21. P. Rondou, G. Haegeman, P. Vanhoenacker, and C.K. Van BTB Protein KLHL12 targets the dopamine D4 receptor for ubiquitination by a Cul3-based E3 ligase J. Biol. Chem. 283 2008 11083 11096
22. Y. Kigoshi, F. Tsuruta, and T. Chiba Ubiquitin ligase activity of Cul3-KLHL7 protein is attenuated by autosomal dominant retinitis pigmentosa causative mutation J. Biol. Chem. 286 2011 33613 33621
23. Y. Jiang, W.B. Ferguson, and J.B. Peng WNK4 enhances TRPV5-mediated calcium transport: potential role in hypercalciuria of familial hyperkalemic hypertension caused by gene mutation of WNK4 Am. J. Physiol. Renal Physiol. 292 2007 F545 F554
24. W. Zhang, T. Na, G. Wu, H. Jing, and J.B. Peng Down-regulation of intestinal apical calcium entry channel TRPV6 by ubiquitin E3 ligase Nedd4-2 J. Biol. Chem. 285 2010 36586 36596
25. - cotransporter Am. J. Physiol. Renal Physiol. 295 2008 F1199 F1206
26. M.Z. Hossain Khan, E. Sohara, and A. Ohta Phosphorylation of Na-Cl cotransporter by OSR1 and SPAK kinases regulates its ubiquitination Biochem. Biophys. Res. Commun. 425 2012 456 461
27. D.D. Zhang, S.C. Lo, J.V. Cross, D.J. Templeton, and M. Hannink Keap1 is a redox-regulated substrate adaptor protein for a Cul3-dependent ubiquitin ligase complex Mol. Cell. Biol. 24 2004 10941 10953
28. E.S. Zimmerman, B.A. Schulman, and N. Zheng Structural assembly of cullin-RING ubiquitin ligase complexes Curr. Opin. Struct. Biol. 20 2010 714 721
29. A. Ohta, F.R. Schumacher, and Y. Mehellou The CUL3-KLHL3 E3 ligase complex mutated in Gordon's hypertension syndrome interacts with and ubiquitylates WNK isoforms: disease-causing mutations in KLHL3 and WNK4 disrupt interaction Biochem. J. 451 2013 111 122
30. M. Wakabayashi, T. Mori, and K. Isobe Impaired KLHL3-mediated ubiquitination of WNK4 causes human hypertension Cell Rep. 3 2013 1 11