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. 2012 Nov 20;104(22):1750–1764. doi: 10.1093/jnci/djs418

Figure 2.

Figure 2.

Mitochondrial function in FLCN-deficient muscle tissues. A) Relative tricarboxylic acid cycle metabolite values for citrate and malate were obtained in metabolite profiling analyses on quadriceps muscle tissues of indicated genotypes. Mean ± 95% confidence interval. Quadriceps, n = 6. Student’s t test (two-sided). B) Coenzymes for the electron transport chain were measured in quadriceps muscle tissues of indicated genotypes. Nicotinamide adenine dinucleotide, reduced (NADH) was measured using a commercially available kit, and a relative value of flavin adenine dinucleotide was obtained by metabolite profiling analyses. Mean ± 95% confidence interval. Quadriceps, n = 6. Student’s t test (two-sided). C) Respiratory capacity of isolated mitochondria was analyzed in muscle tissues of indicated genotypes. State 3 respiration of complex I– and complex IV–dependent respiration was measured by Seahorse XF96 analyzer using mitochondria isolated from the entire hind limb muscle. Mean ± 95% confidence interval. n = 4. Student’s t test (two-sided). D) Relative ATP amounts were obtained from metabolite profiling analyses on quadriceps muscle tissues of indicated genotypes. Mean ± 95% confidence interval. Quadriceps, n = 6. Student’s t test (two-sided).