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Familial Hyperkalemic Hypertension.
Compr Physiol
December 2024
Division of Nephrology and Hypertension, Department of Medicine, Oregon Health and Science University, Portland, Oregon, USA.
The rare disease Familial Hyperkalemic Hypertension (FHHt) is caused by mutations in the genes encoding Cullin 3 (CUL3), Kelch-Like 3 (KLHL3), and two members of the With-No-Lysine [K] (WNK) kinase family, WNK1 and WNK4. In the kidney, these mutations ultimately cause hyperactivation of NCC along the renal distal convoluted tubule. Hypertension results from increased NaCl retention, and hyperkalemia by impaired K secretion by downstream nephron segments.
View Article and Find Full Text PDFMacrophage SPAK deletion limits a low potassium-induced kidney inflammatory program.
Am J Physiol Renal Physiol
November 2024
Division of Nephrology, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, United States.
Inadequate dietary potassium (K) consumption is a significant contributor to poor cardiovascular outcomes. A diet with reduced K content has been shown to cause salt-sensitive increases in blood pressure. More recently, we have also shown that reductions in blood K can cause direct kidney injury, independent of dietary sodium (Na) content.
View Article and Find Full Text PDFDistal convoluted tubule-specific disruption of the COP9 signalosome but not its regulatory target cullin 3 causes tubular injury.
Am J Physiol Renal Physiol
October 2024
Division of Nephrology and Hypertension, Department of Medicine, Oregon Health and Science University, Portland, Oregon, United States.
Article Synopsis
- Familial hyperkalemic hypertension (FHHt), also known as Gordon syndrome, results from abnormal WNK4 accumulation that activates the NaCl cotransporter (NCC) in the kidneys, primarily affecting the distal convoluted tubule (DCT).
- Mutations in the cullin 3 (CUL3) gene disrupt its interaction with the COP9 signalosome, leading to WNK4 accumulation and potential kidney injury, but short-term experiments show no significant plasma electrolyte changes in DCT-specific knockout mice.
- Long-term DCT-specific deletion of CUL3 causes kidney injury and atrophy, indicating that CUL3's role in degrading WNK4 is crucial for preventing FHHt, highlighting how the
With No Lysine (K) Kinases and Sodium Transporter Function in Solute Exchange with Implications for BP Regulation as Elucidated through Drosophila.
Kidney360
October 2024
Molecular Medicine Program, University of Utah, Salt Lake City, Utah; Division of Nephrology and Hypertension, Department of Internal Medicine, University of Utah, Salt Lake City, Utah; Department of Human Genetics, University of Utah, Salt Lake City, Utah; and Medical Service, Veterans Affairs Salt Lake City Health Care System, Salt Lake City, Utah.
Like other multicellular organisms, the fruit fly Drosophila melanogaster must maintain homeostasis of the internal milieu, including the maintenance of constant ion and water concentrations. In mammals, the with no lysine (K) (WNK)-Ste20-proline/alanine rich kinase/oxidative stress response 1 kinase cascade is an important regulator of epithelial ion transport in the kidney. This pathway regulates SLC12 family cotransporters, including sodium-potassium-2-chloride, sodium chloride, and potassium chloride cotransporters.
View Article and Find Full Text PDFRegulating distal nephron functions and salt sensitivity.
Am J Physiol Renal Physiol
October 2024
Department of Clinical Laboratory, School of Medicine, International University of Health and Welfare, Narita, Japan.
This review highlights the molecular basis of salt sensitivity in hypertension, with a focus on the regulation of sodium transport in the distal nephron. Sodium reabsorption in this region is often linked to the actions of aldosterone, although in recent years numerous findings have been reported on the aldosterone-independent pathway of acquiring salt sensitivity by potassium deficiency or potassium loading. The key to this discussion is the interplay, through extracellular potassium concentration, between the first part of the tubules expressing the Na-Cl cotransporter (NCC) and the second part expressing the epithelial Na channel (ENaC).
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