It presents with hypokalaemia, metabolic alkalosis, salt-sensitive hypertension, suppression of plasma renin activity(PRA) and aldosterone secretion.12 Gordon syndrome also known as pseudohypoaldosteronism type II is caused by mutations in any of the four genes; WNK1, WNK4, CUL3
and KLHL3 which are the regulators of the thiazide sensitive Na+- Cl- co transporter.
To the Editor: Pseudohypoaldosteronism Type II (PHAII), also known as Gordon syndrome, is a rare autosomal disease, caused by mutations in WNK1, WNK4, CUL3
, or KLHL3 genes.
According to the human reference genome (GRCh37/hg19) from the Ensembl genome database (http://www.ensembl.org), hsa_circ_0008309 is located at chr2: 225400244-225422573 and the parental gene is Cullin 3 (CUL3
Mendelian forms of hypertension and germline mutations causing early-onset hypertension have highlighted biological pathways that involve renal salt handling (WNK1, WNK4, KLHL3, and CUL3
), ion transport (CACNA1D, CACNA1H, KCNJ5, SCNN1B, and SCNN1G), corticosteroidogenesis (CYP11B2, HSD11B2, NR3C2, CYP11B1, and CYP17A1), and vascular tone (PDE3A) to regulate blood [41-44].
Padmanabhan, "Analysis of dimerization of BTB-IVR domains of Keap1 and its interaction with Cul3
, by molecular modeling," Bioinformation, vol.
Functional activity of Nrf2 strongly depends on its regulation by the Keapl adaptor protein, in the absence of electrophiles or oxidants, Keapl binds to and sequesters Nrf2 in the cytoplasm, resulting in Nrf2 ubiquitination by Cul3
and degradation in the proteasome.
(2009) The human Dcn1-like protein DCNL3 promotes Cul3
neddylation at membranes.
Two proteins, CUL3
and EP300, were identified to be hub molecules, with degrees of 56 and 21, respectively.
Loss of Cul1 or of the F-box protein Slimb represses arborization, while loss of Cul3
enhances it, suggesting that theCul1 andCul3 E3 complexes target proteins withmarkedly different roles in dendritogenesis.
Cysteine-based regulation of the CUL3
adaptor protein Keap1.
The order of thermal stabilities these compounds were found: CuL4 > CuL1 > CuL6 > CuL2, 5 > CuL3
> L5 > L1, 2, 4> L3 > L6, which may be attributed to the specific chemical structures of the synthesized ligands and copper(II) complexes.
) is the core component of multiple cullin-RING-based BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complexes which mediate the ubiquitination and subsequent proteasomal degradation of target proteins .