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Kelch-like 3

KLHL3
This gene is ubiquitously expressed and encodes a full-length protein which has an N-terminal BTB domain followed by a BACK domain and six kelch-like repeats in the C-terminus. These kelch-like repeats promote substrate ubiquitination of bound proteins via interaction of the BTB domain with the CUL3 (cullin 3) component of a cullin-RING E3 ubiquitin ligase (CRL) complex. Muatations in this gene cause pseudohypoaldosteronism type IID (PHA2D); a rare Mendelian syndrome featuring hypertension, hyperkalaemia and metabolic acidosis. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Mar 2012] (from NCBI)
Top mentioned proteins: Cul3, WNK4, Ubiquitin, Cullin, CAN
Papers on KLHL3
Degradation by Cullin 3 and effect on WNK kinases suggest a role of KLHL2 in the pathogenesis of Familial Hyperkalemic Hypertension.
New
Yang et al., Shanghai, China. In Biochem Biophys Res Commun, Feb 2016
Mutations in WNK1 and WNK4, and in components of the Cullin-Ring Ligase system, kelch-like 3 (KLHL3) and Cullin 3 (CUL3), can cause the rare hereditary disease, Familial Hyperkalemic Hypertension (FHHt).
Involvement of selective autophagy mediated by p62/SQSTM1 in KLHL3-dependent WNK4 degradation.
New
Uchida et al., Tokyo, Japan. In Biochem J, Dec 2015
We reported that kelch-like protein 3 (KLHL3)-Cullin3 E3 ligase ubiquitinates with-no-lysine kinase 4 (WNK4) and that impaired WNK4 ubiquitination causes pseudohypoaldosteronism type II, a hereditary hypertensive disease.
Impaired degradation of WNK by Akt and PKA phosphorylation of KLHL3.
New
Sohara et al., Tokyo, Japan. In Biochem Biophys Res Commun, Dec 2015
Mutations in with-no-lysine kinase (WNK) 1, WNK4, Kelch-like 3 (KLHL3), and Cullin3 result in an inherited hypertensive disease, pseudohypoaldosteronism type II.
Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia.
New
Kurz et al., Zürich, Switzerland. In Embo Mol Med, Oct 2015
Bound to KLHL3, CUL3-RBX1 ubiquitylates WNK kinases, promoting their ubiquitin-mediated proteasomal degradation.
Structural Insights into KCTD Protein Assembly and Cullin3 Recognition.
New
Privé et al., Toronto, Canada. In J Mol Biol, Oct 2015
Previous crystal structures of the homodimeric BTB proteins KLHL3, KLHL11 and SPOP in complex with the N-terminal domain of Cul3 revealed the features required for Cul3 recognition in these proteins.
Kelch-like 3/Cullin 3 ubiquitin ligase complex and WNK signaling in salt-sensitive hypertension and electrolyte disorder.
Review
New
Uchida et al., Tokyo, Japan. In Nephrol Dial Transplant, Aug 2015
Two additional genes responsible for PHAII, Kelch-like 3 (KLHL3) and Cullin 3 (CUL3), were identified in 2012.
6C.01: CULLIN-3 MUTATIONS LEADING TO SKIPPING OF EXON 9 ARE RESPONSIBLE FOR SEVERE CASES OF FAMILIAL HYPERKALAEMIC HYPERTENSION.
New
Jeunemaitre et al., Rennes, France. In J Hypertens, Jun 2015
In 2012, an American laboratory as well as ours, have identified two other genes, KLHL3 and CUL3 as responsible for the disease.
1A.01: MUTATIONS AFFECTING THE CONSERVED ACIDIC MOTIF OF WNK1 CAUSE INHERITED NORMOTENSIVE HYPERKALEMIC ACIDOSIS.
New
Jeunemaitre et al., London, United Kingdom. In J Hypertens, Jun 2015
More recently, mutations in the KLHL3-CUL3 E3 ubiquitin ligase complex have shed light on the importance of the With-No-Lysine kinases (WNKs) cellular degradation on ion transport.
Revisiting the NaCl cotransporter regulation by with-no-lysine kinases.
Review
New
Gamba et al., Mexico. In Am J Physiol Cell Physiol, Jun 2015
Two genes encode for with-no-lysine (K) kinases WNK1 and WNK4, while two encode for kelch-like 3 (KLHL3) and cullin 3 (CUL3) proteins that form a RING type E3 ubiquitin ligase complex.
Generation and analysis of knock-in mice carrying pseudohypoaldosteronism type II-causing mutations in the cullin 3 gene.
Uchida et al., Tokyo, Japan. In Biol Open, 2014
Pseudohypoaldosteronism type II (PHAII) is a hereditary hypertensive disease caused by mutations in four different genes: with-no-lysine kinases (WNK) 1 and 4, Kelch-like family member 3 (KLHL3), and cullin 3 (Cul3).
Regulation of blood pressure and renal electrolyte balance by Cullin-RING ligases.
Review
Uchida, Tokyo, Japan. In Curr Opin Nephrol Hypertens, 2014
We here discuss a novel pathogenic mechanism underlying the hereditary hypertensive disease pseudohypoaldosteronism type II (PHAII), caused by mutations in three different genes encoding for Cullin-3, Kelch-like protein 3 (KLHL3), and with-no-lysine kinases (WNKs).
Insights in cullin 3/WNK4 and its relationship to blood pressure regulation and electrolyte homeostasis.
Review
Pedraza-Chaverri et al., Mexico. In Cell Signal, 2014
Recently, Cul3 has been linked to the development of type II pseudohypoaldosteronism (PHAII or Gordon's syndrome) due to the fact that Cul3 has the ability to bind to Kelch-like 3 protein (KLHL3) and therefore mediating the degradation of some members of the WNK kinases.
Regulation of with-no-lysine kinase signaling by Kelch-like proteins.
Review
Sasaki et al., Tokyo, Japan. In Biol Cell, 2014
In 2012, two additional genes responsible for PHAII, Kelch-like 3 (KLHL3) and Cullin3, were identified.
KLHL3 mutations cause familial hyperkalemic hypertension by impairing ion transport in the distal nephron.
Impact
GeneRIF
Jeunemaitre et al., Paris, France. In Nat Genet, 2012
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
Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities.
Impact
GeneRIF
Lifton et al., New Haven, United States. In Nature, 2012
fundamental role for KLHL3 and CUL3 in blood pressure, K(+) and pH homeostasis
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