Oxidants and Cardiorenal Vascular Remodeling-Insights from Rare Genetic Tubulopathies: Bartter's and Gitelman's Syndromes

• Published in: Antioxidants Vol. 12; no. 4; p. 811
• Main Authors: Sgarabotto, Luca, Ravarotto, Verdiana, Stefanelli, Lucia Federica, Cacciapuoti, Martina, Davis, Paul A, Nalesso, Federico, Calò, Lorenzo A
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 26-03-2023; MDPI
• Subjects: Angiotensin; Angiotensin II; Bartter’s syndrome; Blood pressure; cardiovascular remodeling; Electrolytes; Failure to thrive; Free radicals; Genetic aspects; Gitelman’s syndrome; Heart; Hyperaldosteronism; Hyperplasia; Hypertension; Hypokalemia; Hypotension; Kidneys; Kinases; Mutation; Nitric oxide; Opinion; Oxidants; Oxidative stress; Permeability; Polyuria; Proteins; renal remodeling; Signal transduction; Smooth muscle; United States; renal remodeling; Bartter’s syndrome; Gitelman’s syndrome; oxidants; cardiovascular remodeling
• ISSN: 2076-3921; 2076-3921
• DOI: 10.3390/antiox12040811

Two human genetic tubulopathies, Bartter's (BS) and Gitelman's (GS) syndromes, have normo/hypotension and absent cardiac remodeling despite their apparent angiotensin system (RAS) activation. This seeming contradiction has led to an extensive investigation of BSGS patients, the result of which is that BSGS represents a mirror image of hypertension. BSGS's unique set of properties has then permitted their use as a human model to probe and characterize RAS system pathways and oxidative stress in cardiovascular and renal remodeling and pathophysiology. This review details the results using GSBS patients that provide a deeper understanding of Ang II signaling and its associated oxidants/oxidative stress in humans. By providing a more complete and complex picture of cardiovascular and renal remodeling pathways and processes, studies of GSBS can inform the identification and selection of new targets and therapies to treat these and other oxidant-related disorders.