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. Author manuscript; available in PMC: 2009 Aug 25.
Published in final edited form as: FASEB J. 2008 Jan 29;22(6):1874–1885. doi: 10.1096/fj.07-100149

Figure 4.

Figure 4

Adenine nucleotide levels. In galactose medium, steady-state levels of total adenine nucleotides (ANs) and ATP were not significantly decreased in P1 but were reduced in P2 and P3 (A, B). ATP/ADP ratio, a value that reflects the state of mitochondrial ATP production, was significantly decreased in the cells from all three patients (P<0.05), but the decreases were more dramatic in P2 and P3 than P1 fibroblasts (C). In glucose-rich media, P2 but not P1 cells showed decreased ATP/ADP ratio, whereas neither P1 nor P2 fibroblasts showed significant decreases in total ANs or ATP (D,E). ANs were measured in P1 cells grown in media supplemented with 10% dialyzed FBS and demonstrated decreased levels of ATP, ATP/ADP ratio, and AN compared with cells cultured in galactose or glucose-rich media, indicating that glucose in undialyzed FBS provided substrate for anaerobic glycolysis (A-C). Control (C) data are expressed as the mean ± SD of 5 different samples in triplicate experiments. Patient data are expressed as the mean ± SD of triplicate experiments.*P < 0.05 vs. control; **P < 0.01 vs. control.