KLHL3-dependent WNK4 degradation affected by potassium through the neddylation and autophagy pathway. | KLHL3-dependent WNK4 degradation affected by potassium through the neddylation and autophagy pathway. Ying S, Guo Q, Zhang C., Free PMC Article | 07/27/2023 |
KLHL3 deficiency in mice ameliorates obesity, insulin resistance, and nonalcoholic fatty liver disease by regulating energy expenditure. | KLHL3 deficiency in mice ameliorates obesity, insulin resistance, and nonalcoholic fatty liver disease by regulating energy expenditure. Jang JH, Lee JW, Cho MJ, Hwang B, Kwon MG, Kim DH, Lee NK, Lee J, Park YJ, Yang YR, Kim J, Kim YH, An TH, Oh KJ, Bae KH, Park JG, Min JK., Free PMC Article | 09/17/2022 |
Generation and analysis of pseudohypoaldosteronism type II knock-in mice caused by a nonsense KLHL3 mutation in the Kelch domain. | Generation and analysis of pseudohypoaldosteronism type II knock-in mice caused by a nonsense KLHL3 mutation in the Kelch domain. Lin CM, Sung CC, Yang SS, Chen YC, Huang SM, Lin SH. | 06/18/2022 |
Combined Kelch-like 3 and Cullin 3 Degradation is a Central Mechanism in Familial Hyperkalemic Hypertension in Mice. | Combined Kelch-like 3 and Cullin 3 Degradation is a Central Mechanism in Familial Hyperkalemic Hypertension in Mice. Maeoka Y, Ferdaus MZ, Cornelius RJ, Sharma A, Su XT, Miller LN, Robertson JA, Gurley SB, Yang CL, Ellison DH, McCormick JA., Free PMC Article | 04/30/2022 |
Decreased KLHL3 expression is involved in the activation of WNK-OSR1/SPAK-NCC cascade in type 1 diabetic mice. | Decreased KLHL3 expression is involved in the activation of WNK-OSR1/SPAK-NCC cascade in type 1 diabetic mice. Guo Q, Zhang Y, Jiang GR, Zhang C. | 12/18/2021 |
Role of KLHL3 and dietary K(+) in regulating KS-WNK1 expression. | Role of KLHL3 and dietary K(+) in regulating KS-WNK1 expression. Ostrosky-Frid M, Chávez-Canales M, Zhang J, Andrukhova O, Argaiz ER, Lerdo-de-Tejada F, Murillo-de-Ozores A, Sanchez-Navarro A, Rojas-Vega L, Bobadilla NA, Vazquez N, Castañeda-Bueno M, Alessi DR, Gamba G., Free PMC Article | 05/15/2021 |
Klhl3(M131V/+) KI mice feature typical pseudohypoaldosteronism II with a simultaneous increase of WNK1 and WNK4 through the impaired KLHL3 BTB domain binding to Cul3 | Generation and analysis of a mouse model of pseudohypoaldosteronism type II caused by KLHL3 mutation in BTB domain. Lin CM, Cheng CJ, Yang SS, Tseng MH, Yen MT, Sung CC, Lin SH. | 09/21/2019 |
deletion of exon 9 from Cul3 generates a protein that is itself ubiquitin-ligase defective but also capable of enhanced autophagocytic KLHL3 degradation | Dual gain and loss of cullin 3 function mediates familial hyperkalemic hypertension. Cornelius RJ, Zhang C, Erspamer KJ, Agbor LN, Sigmund CD, Singer JD, Yang CL, Ellison DH., Free PMC Article | 09/7/2019 |
decreased abundance of KLHL3 is a specific phenomenon caused by mutant CUL3 (Deltaexon9). | Decreased KLHL3 expression is involved in the pathogenesis of pseudohypoaldosteronism type II caused by cullin 3 mutation in vivo. Yoshida S, Araki Y, Mori T, Sasaki E, Kasagi Y, Isobe K, Susa K, Inoue Y, Bomont P, Okado T, Rai T, Uchida S, Sohara E. | 03/9/2019 |
However, phosphorylation of SPAK and NCC at distal convoluted tubules were almost completely absent even in WNK4(-/-)KLHL3(R528H/R528H) mice. In conclusion, increased WNK1 was unable to compensate for WNK4 deficiency and phosphorylate the NCC, indicating that WNK4 is indispensable for the onset of PHAII. | WNK4 is indispensable for the pathogenesis of pseudohypoaldosteronism type II caused by mutant KLHL3. Susa K, Sohara E, Takahashi D, Okado T, Rai T, Uchida S. | 09/16/2017 |
the heterozygous deletion of KLHL3 was not sufficient to cause PHAII, indicating that autosomal dominant type pseudohypoaldosteronism type II (PHAII) is caused by the dominant negative effect of mutant KLHL3. | KLHL3 Knockout Mice Reveal the Physiological Role of KLHL3 and the Pathophysiology of Pseudohypoaldosteronism Type II Caused by Mutant KLHL3. Sasaki E, Susa K, Mori T, Isobe K, Araki Y, Inoue Y, Yoshizaki Y, Ando F, Mori Y, Mandai S, Zeniya M, Takahashi D, Nomura N, Rai T, Uchida S, Sohara E., Free PMC Article | 07/1/2017 |
Potassium depletion stimulates NCC via phosphorylation and inactivation of the KLHL3 and promoting increased blood pressure. | Potassium depletion stimulates Na-Cl cotransporter via phosphorylation and inactivation of the ubiquitin ligase Kelch-like 3. Ishizawa K, Xu N, Loffing J, Lifton RP, Fujita T, Uchida S, Shibata S., Free PMC Article | 05/20/2017 |
KLHL3 regulates paracellular chloride transport in the kidney by ubiquitination of claudin-8 | KLHL3 regulates paracellular chloride transport in the kidney by ubiquitination of claudin-8. Gong Y, Wang J, Yang J, Gonzales E, Perez R, Hou J., Free PMC Article | 07/4/2015 |
increased protein expression levels of WNK1 and WNK4 kinases cause PHAII by KLHL3 R528H mutation due to impaired KLHL3-Cullin3-mediated ubiquitination. | Impaired degradation of WNK1 and WNK4 kinases causes PHAII in mutant KLHL3 knock-in mice. Susa K, Sohara E, Rai T, Zeniya M, Mori Y, Mori T, Chiga M, Nomura N, Nishida H, Takahashi D, Isobe K, Inoue Y, Takeishi K, Takeda N, Sasaki S, Uchida S. | 05/30/2015 |