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10.15252/emmm.201505444 http://scihub22266oqcxt.onion/10.15252/emmm.201505444 26286618!4604684!26286618     free     free     free Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 265.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26286618.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       EMBO+Mol+Med 2015 ; 7 (10): 1285-306 Nephropedia Template TP {{tp|p=26286618|t=2015. Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia |pdf=|usr=}}{{26286618}} gab.com Text Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia JO: EMBO Mol Med http://www.kidney.de/pget.php?&tw=1&pmid=26286618 #FOAvip Deletion of exon 9 from Cullin-3 (CUL3, residues 403-459: CUL3(Delta403-459)) causes pseudohypoaldosteronism type IIE (PHA2E), a severe form of familial hyperkalaemia and hypertension (FHHt). CUL3 binds the RING protein RBX1 and various substrate adaptors to form Cullin-RING-ubiquitin-ligase complexes. Bound to KLHL3, CUL3-RBX1 ubiquitylates WNK kinases, promoting their ubiquitin-mediated proteasomal degradation. Since WNK kinases activate Na/Cl co-transporters to promote salt retention, CUL3 regulates blood pressure. Mutations in both KLHL3 and WNK kinases cause PHA2 by disrupting Cullin-RING-ligase formation. We report here that the PHA2E mutant, CUL3(Delta403-459), is severely compromised in its ability to ubiquitylate WNKs, possibly due to altered structural flexibility. Instead, CUL3(Delta403-459) auto-ubiquitylates and loses interaction with two important Cullin regulators: the COP9-signalosome and CAND1. A novel knock-in mouse model of CUL3(WT) (/Delta403-459) closely recapitulates the human PHA2E phenotype. These mice also show changes in the arterial pulse waveform, suggesting a vascular contribution to their hypertension not reported in previous FHHt models. These findings may explain the severity of the FHHt phenotype caused by CUL3 mutations compared to those reported in KLHL3 or WNK kinases. Twit Text FOAVip Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia ????EMBO Mol Med http://www.kidney.de/pget.php?&tw=1&pmid=26286618 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26286618 #FOAvip English Wikipedia <ref>{{Cite pmid|26286618}}</ref> Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia #MMPMID26286618 Schumacher FR; Siew K; Zhang J; Johnson C; Wood N; Cleary SE; Al Maskari RS; Ferryman JT; Hardege I; Yasmin; Figg NL; Enchev R; Knebel A; O'Shaughnessy KM; Kurz T EMBO Mol Med 2015[Oct]; 7 (10): 1285-306 PMID26286618show ga Deletion of exon 9 from Cullin-3 (CUL3, residues 403-459: CUL3(Delta403-459)) causes pseudohypoaldosteronism type IIE (PHA2E), a severe form of familial hyperkalaemia and hypertension (FHHt). CUL3 binds the RING protein RBX1 and various substrate adaptors to form Cullin-RING-ubiquitin-ligase complexes. Bound to KLHL3, CUL3-RBX1 ubiquitylates WNK kinases, promoting their ubiquitin-mediated proteasomal degradation. Since WNK kinases activate Na/Cl co-transporters to promote salt retention, CUL3 regulates blood pressure. Mutations in both KLHL3 and WNK kinases cause PHA2 by disrupting Cullin-RING-ligase formation. We report here that the PHA2E mutant, CUL3(Delta403-459), is severely compromised in its ability to ubiquitylate WNKs, possibly due to altered structural flexibility. Instead, CUL3(Delta403-459) auto-ubiquitylates and loses interaction with two important Cullin regulators: the COP9-signalosome and CAND1. A novel knock-in mouse model of CUL3(WT) (/Delta403-459) closely recapitulates the human PHA2E phenotype. These mice also show changes in the arterial pulse waveform, suggesting a vascular contribution to their hypertension not reported in previous FHHt models. These findings may explain the severity of the FHHt phenotype caused by CUL3 mutations compared to those reported in KLHL3 or WNK kinases. |*Disease Models, Animal[MESH] |*Mutation[MESH] |Animals[MESH] |Cullin Proteins/*genetics/metabolism[MESH] |HEK293 Cells[MESH] |Humans[MESH] |Intracellular Signaling Peptides and Proteins/metabolism[MESH] |Mice[MESH] |Mice, Knockout[MESH] |Protein Serine-Threonine Kinases/metabolism[MESH]Characterisation of the Cullin-3 mutation that causes a severe form of(epmc).pdf DeepDyve Pubget Overpricing