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Article

Distal convoluted tubule-specific disruption of the COP9 signalosome but not its regulatory target cullin 3 causes tubular injury.

American journal of physiology. Renal physiology - 1 Oct 2024

Maeoka Yujiro, Bradford Tanner, Su Xiao-Tong, Sharma Avika, Yang Chao-Ling, Ellison David H, McCormick James A, Cornelius Ryan J

Abstract excerpt

The disease familial hyperkalemic hypertension (FHHt; also known as Gordon syndrome) is caused by aberrant accumulation of with-no-lysine kinase (WNK4) activating the NaCl cotransporter (NCC) in the distal convoluted tubule (DCT) of the kidney. Mutations in cullin 3 (CUL3) cause FHHt by disrupting interaction with the deneddylase COP9 signalosome (CSN). Deletion of Cul3 or Jab1 (the catalytically active CSN...

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Identifiers

PMID: 39205661
DOI: 10.1152/ajprenal.00138.2024
PMCID: PMC11483082

Genes

DCT
WNK4
CUL3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

Related research

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Research trail→

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Distal convoluted tubule-specific disruption of the COP9 signalosome but not its regulatory target cullin 3 causes tubular injury.PMID 39205661Read ↗

Hypertension-causing cullin 3 mutations disrupt COP9 signalosome binding.Semantic proximity Read ↗

Mutant Cullin 3 causes familial hyperkalemic hypertension via dominant effects.Semantic proximity Read ↗

Renal effects of cullin 3 mutations causing familial hyperkalemic hypertension.Semantic proximity Read ↗

Regulation of the renal NaCl cotransporter by the WNK/SPAK pathway: lessons learned from genetically altered animals.Semantic proximity Read ↗

Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities.Semantic proximity Read ↗

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ROMK expression remains unaltered in a mouse model of familial hyperkalemic hypertension caused by the CUL3Δ403-459 mutation.Semantic proximity Read ↗

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KLHL3 mutations cause familial hyperkalemic hypertension by impairing ion transport in the distal nephron.Semantic proximity Read ↗

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