Data di Pubblicazione:
2018
Citazione:
Renal COP9 signalosome deficiency alters CUL3-KLHL3-WNK signaling pathway / Cornelius, R. J.; Si, J.; Cuevas, C. A.; Nelson, J. W.; Gratreak, B. D. K.; Pardi, R.; Yang, C. -L.; Ellison, D. H.. - In: JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY. - ISSN 1046-6673. - 29:11(2018), pp. 2627-2640. [10.1681/ASN.2018030333]
Abstract:
The familial hyperkalemic hypertension (FHHt) cullin 3 (CUL3) mutant does not degrade WNK kinases normally, thereby leading to thiazide-sensitive Na-Cl cotransporter (NCC) activation. CUL3 mutant (CUL3Δ9) does not bind normally to the COP9 signalosome (CSN), a deneddylase involved in regulating cullin-RING ligases. CUL3Δ9 also caused increased degradation of the CUL3-WNK substrate adaptor kelch-like 3 (KLHL3). Here, we sought to determine how defective CSN action contributes to the CUL3Δ9 phenotype.
Tipologia CRIS:
1.1 Articolo in rivista
Keywords:
Cell & Transport Physiology; Na transport; distal tubule; renal hypertension; Animals; COP9 Signalosome Complex; Cullin Proteins; Disease Models, Animal; Female; HEK293 Cells; Humans; Intracellular Signaling Peptides and Proteins; Kidney; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Microfilament Proteins; Microscopy, Fluorescence; Mutation; Nephrons; Peptide Hydrolases; Phenotype; Protein-Serine-Threonine Kinases; Proteolysis; Pseudohypoaldosteronism; Signal Transduction
Elenco autori:
Cornelius, R. J.; Si, J.; Cuevas, C. A.; Nelson, J. W.; Gratreak, B. D. K.; Pardi, R.; Yang, C. -L.; Ellison, D. H.
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