ReviewPediatric nephrology (Berlin, Germany)2015
Gordon Syndrome: a continuing story.
Review in Pediatric nephrology (Berlin, Germany), 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
35 citing papers in PubMed, 84 citations in OpenAlex.
- Monogenic Kidney Disorders as Hidden Drivers of Electrolyte Imbalance and Hypertension.Electrolyte & blood pressure : E & BP · 2026Review
- Exploring the Role of the SLC12A Cation-Coupled Chloride Cotransporters in the Cardiovascular System.Cells · 2026Review
- Salt and chronic kidney disease.Nature reviews. Nephrology · 2026Review
- [Consensus recommendations on the diagnosis and treatment of hyperkalemia of the Austrian Society of Nephrology 2026].Wiener klinische Wochenschrift · 2026Review
- Do Not Dismiss Incidental Hyperkalemia in Childhood: Early Recognition of Pseudohypoaldosteronism Type II.Cureus · 2026Article
- Pathophysiology and causes of hyperkalemia: unraveling causes beyond kidney dysfunction.Clinical and experimental nephrology · 2025Review
- Adrenal causes of endocrine hypertension in childhood or adolescence.Journal of endocrinological investigation · 2025Review
- KS-WNK1 augments the effects of dietary potassium intake on renal sodium chloride reabsorption.The Journal of clinical investigation · 2025Article
- Article
- Hyperkalaemic acidosis: blood pressure is the diagnostic clue.Pediatric nephrology (Berlin, Germany) · 2025Article
- MAGED2 Enhances Expression and Function of NCC at the Cell Surface via cAMP Signaling Under Hypoxia.Cells · 2025Article
- Pseudohypoaldosteronism type II: The Relevance of A Challenging Diagnosis.European journal of case reports in internal medicine · 2025Article
- Navigating the multifaceted intricacies of the NaPhysiological reviews · 2024Review
- Article
- Article
- Gordon's syndrome in pregnancy.Obstetric medicine · 2023Review
- Urinary extracellular vesicle mRNA analysis of sodium chloride cotransporter in hypertensive patients under different conditions.Journal of human hypertension · 2023Article
- Kidney and blood pressure regulation-latest evidence for molecular mechanisms.Clinical kidney journal · 2023Review
- Summary of Known Genetic and Epigenetic Modification Contributed to Hypertension.International journal of hypertension · 2023Review
- Monogenic forms of low-renin hypertension: clinical and molecular insights.Pediatric nephrology (Berlin, Germany) · 2022Article
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
Abstract
Gordon Syndrome (GS) is a rare familial hypertension syndrome with a characteristic hyperkalaemia which distinguishes it from other syndromic forms of hypertension that typically cause hypokalaemia. Patients with GS respond to aggressive salt-restriction or relatively small doses of thiazide diuretics, which suggests that activation of the thiazide-sensitive Na/Cl cotransporter (NCC) in the distal nephron is to blame. However, the mechanism has proved to be complex. In 2001, mutations in genes encoding two serine/threonine kinases, WNK1 and WNK4, were identified as causing GS. However, it took several years to appreciate that these kinases operated in a cascade with downstream serine/threonine kinases (SPAK and OSR1) actually phosphorylating and activating NCC and the closely related cotransporters NKCC1 and NKCC2. The hyperkalaemia in GS arises from an independent action of WNK1/WNK4 to reduce cell-surface expression of ROMK, the secretory K-channel in the collecting ducts. However, mutations in WNK1/4 are present in a small minority of GS families, and further genes have emerged (CUL3 and KLHL3) that code for Cullin-3 (a scaffold protein in an ubiquitin-E3 ligase) and an adaptor protein, Kelch3, respectively. These new players regulate the ubiquitination and proteasomal degradation of WNK kinases, thereby adding to the complex picture we now have of NCC regulation in the distal nephron.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.