Mitochondrial Dysfunction in Kidney Tubulopathies

• Published in: Annual review of physiology 2024-02, Vol.86 (1), p.379-403
• Main Authors: Hoogstraten, Charlotte A, Hoenderop, Joost G, de Baaij, Jeroen H.F
• Format: Article
• Language: English
• Subjects: Acidosis; Apoptosis; Autophagy; Bartter syndrome; Bartter Syndrome - metabolism; Bartter Syndrome - pathology; Cellular stress response; Disorders; Distal tubules; Fanconi syndrome; Gitelman syndrome; Humans; Kearns-Sayre syndrome; Kearns-Sayre Syndrome - metabolism; Kearns-Sayre Syndrome - pathology; Kidney Diseases - pathology; Kidney Tubules - metabolism; Kidney Tubules - pathology; Kidneys; Leigh syndrome; Mitochondria; mitochondrial cytopathy; Proximal tubules; Reabsorption; Renal function; Renal tubular acidosis; renal tubulopathy; Tubules
• ISSN: 0066-4278; 1545-1585
• DOI: 10.1146/annurev-physiol-042222-025000

Mitochondria play a key role in kidney physiology and pathology. They produce ATP to fuel energy-demanding water and solute reabsorption processes along the nephron. Moreover, mitochondria contribute to cellular health by the regulation of autophagy, (oxidative) stress responses, and apoptosis. Mitochondrial abundance is particularly high in cortical segments, including proximal and distal convoluted tubules. Dysfunction of the mitochondria has been described for tubulopathies such as Fanconi, Gitelman, and Bartter-like syndromes and renal tubular acidosis. In addition, mitochondrial cytopathies often affect renal (tubular) tissues, such as in Kearns-Sayre and Leigh syndromes. Nevertheless, the mechanisms by which mitochondrial dysfunction results in renal tubular diseases are only scarcely being explored. This review provides an overview of mitochondrial dysfunction in the development and progression of kidney tubulopathies. Furthermore, it emphasizes the need for further mechanistic investigations to identify links between mitochondrial function and renal electrolyte reabsorption.