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.
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]
Renal COP9 signalosome deficiency alters CUL3-KLHL3-WNK signaling pathway
Pardi R.;
2018-01-01
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.| File | Dimensione | Formato | |
|---|---|---|---|
|
20.500.11768 94612 2627.full.pdf
solo gestori archivio
Tipologia:
PDF editoriale (versione pubblicata dall'editore)
Licenza:
Copyright dell'editore
Dimensione
2.23 MB
Formato
Adobe PDF
|
2.23 MB | Adobe PDF | Visualizza/Apri Richiedi una copia |
I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
