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J. Clin. Med. 2017, 6(9), 90;

Measurement of Respiratory Chain Enzyme Activity in Human Renal Biopsy Specimens

Department of Nephrology, Birmingham Children’s Hospital, Birmingham B4 6NH, UK
Department of Histopathology, Birmingham Children’s Hospital, Birmingham B4 6NH, UK
Neurometabolic Unit, National Hospital for Neurology and Neurosurgery, London WC1N 3BG, UK
Author to whom correspondence should be addressed.
Academic Editor: Mark S. Sands
Received: 6 July 2017 / Revised: 31 August 2017 / Accepted: 13 September 2017 / Published: 19 September 2017
Full-Text   |   PDF [179 KB, uploaded 19 September 2017]


Background: Mitochondrial disorders can present as kidney disease in children and be difficult to diagnose. Measurement of mitochondrial function in kidney tissue may help diagnosis. This study was to assess the feasibility of obtaining renal samples and analysing them for respiratory chain enzyme activity. Methods: The subjects were children undergoing a routine diagnostic renal biopsy, in whom a clinical condition of renal inflammation, scarring and primary metabolic disorder was unlikely. A fresh sample of kidney was snap frozen and later assayed for the activities of respiratory chain enzyme complexes I, II/III, and IV using spectrophotometric enzyme assay, and expressed as a ratio of citrate synthase activity. Results: The range of respiratory chain enzyme activity for complex I was 0.161 to 0.866 (mean 0.404, SD 0.2), for complex II/III was 0.021 to 0.318 (mean 0.177, SD 0.095) and for complex IV was 0.001 to 0.025 (mean 0.015, SD 0.006). There were correlations between the different activities but not between them and the age of the children or a measure of the amount of chronic damage in the kidneys. Conclusion: It is feasible to measure respiratory chain enzyme activity in routine renal biopsy specimens. View Full-Text
Keywords: mitochondrial disorders; renal biopsy; respiratory chain enzymes mitochondrial disorders; renal biopsy; respiratory chain enzymes
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Ghose, A.; Taylor, C.M.; Howie, A.J.; Chalasani, A.; Hargreaves, I.; Milford, D.V. Measurement of Respiratory Chain Enzyme Activity in Human Renal Biopsy Specimens. J. Clin. Med. 2017, 6, 90.

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