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 | |
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