Lookup NU author(s): Professor Robert Taylor
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A multicenter comparison of mitochondrial respiratory chain and complex V enzyme activity tests was performed. The average reproducibility of the enzyme assays is 16% in human muscle samples. In a blinded diagnostic accuracy test in patient fibroblasts and SURF1 knock-out mouse muscle, each lab made the correct diagnosis except for two complex I results. We recommend that enzyme activities be evaluated based on ratios, e.g. with complex IV or citrate synthase activity. In spite of large variations in observed enzyme activities, we show that inter-laboratory comparison of patient sample test results is possible by using normalization against a control sample. (c) 2012 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
Author(s): Rodenburg RJT, Schoonderwoerd GC, Tiranti V, Taylor RW, Rotig A, Valente L, Invernizzi F, Chretien D, He L, Backx GPBM, Janssen KJGM, Chinnery PF, Smeets HJ, De Coo IF, van den Heuvel LP
Publication type: Article
Publication status: Published
Print publication date: 01/01/2013
Online publication date: 17/11/2012
Acceptance date: 07/11/2012
ISSN (print): 1567-7249
ISSN (electronic): 1872-8278
Publisher: Elsevier BV
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