anti-Kelch-Like 3 (Drosophila) (KLHL3) Antibodies

Mouse (Murine) Kelch-Like 3 (Drosophila) (KLHL3) interaction partners

1. 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.

2. 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.

3. Potassium depletion stimulates NCC via phosphorylation and inactivation of the KLHL3 and promoting increased blood pressure.

4. KLHL3 regulates paracellular chloride transport in the kidney by ubiquitination of claudin-8

5. increased protein expression levels of WNK1 and WNK4 kinases cause PHAII by KLHL3 R528H mutation due to impaired KLHL3-Cullin3-mediated ubiquitination.

Human Kelch-Like 3 (Drosophila) (KLHL3) interaction partners

1. A new recessive mutation in KLHL3 (S553L) was identified in familial hyperkalemia and hypertension. Increased urinary NCC was found in affected members (heterozygous) with dominant KLHL3 Q309R, and in affected members (homozygous) of the recessive form.

2. Mutation in the KLHL3 gene is associated with Gordon syndrome.

3. The results demonstrate that Hcy decreases the expression of cMyBP-C through a KLHL3-mediated ubiquitin-proteasome pathway, and thereby influences heart development.

4. This study provides substantial new insights into the role of phosphorylation of KLHL3 in regulating the interaction with WNK4

5. Data indicate that WNK lysine deficient protein kinase 4 protein (WNK4) was degraded not only by proteasomes but also by atypical protein kinase C scaffold protein p62 (p62)-kelch-like 3 protein (KLHL3)-mediated selective autophagy.

6. Familial hyperkalemia and hypertension caused by KLHL3 mutations is accompanied by hypercalciuria as well as hyperkalemia and hypertension.

7. Akt and PKA phosphorylated KLHL3 at S433, and phosphorylation of KLHL3 by PKA inhibited WNK4 degradation.

8. KLHL3 is phosphorylated at serine 433 in the Kelch domain (a site frequently mutated in hypertension with hyperkalemia) by protein kinase C in cultured cells and that this phosphorylation prevents WNK4 binding and degradation.

9. Hyperkalemic hypertension-associated cul3 mutations depletes KLHL3, preventing WNK degradation, despite increased CUL3-mediated WNK ubiquitylation.

10. CUL3 and KLHL3 gene products are physiologically important regulators of thiazide-sensitive distal nephron sodium chloride reabsorption.

11. analysis of how mutations of KLHL3 show less ability to ubiquitinate WNK4 because of KLHL3's low stability and/or decreased binding to CUL3 or WNK4

12. Disease causing mutations in human KLHL3 disrupt the interaction with CUL3, a crystallographic study.

13. KLHL3 is a substrate adaptor for WNK4 in a ubiquitin E3 ligase complex

14. CUL3 and KLHL3 have roles in in electrolyte homeostasis and in Pseudohypoaldosteronism type II

15. 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.

16. identified KLHL3 as a third gene responsible for familial hyperkalemic hypertension; study establishes a role for KLHL3 as a new member of the complex signaling pathway regulating ion homeostasis in the distal nephron and indirectly blood pressure

17. fundamental role for KLHL3 and CUL3 in blood pressure, K(+) and pH homeostasis