Mitochondrial respiratory chain defects present as highly heterogeneous disorders which cannot be unambiguously diagnosed using standard laboratory methods. In this study we observed the biochemical consequences of complex I and complex V deficient human skin fibroblasts when cultivated under galactose stress condition compared to glucose based cell culture condition. We investigated extracellular flux using Seahorse XFe96 cell analyzer and assessed the metabolome fingerprints using High Resolution Magic Angle Spinning NMR. The selective culture method reveals CI and CV defect-specific changes in metabolites associated with the TCA cycle, malate aspartate shuttle and choline metabolism.
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