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. Author manuscript; available in PMC: 2021 Jul 1.
Published in final edited form as: Mol Genet Metab. 2020 May 11;130(3):183–196. doi: 10.1016/j.ymgme.2020.05.003

Figure 3.

Figure 3.

Elevated biochemical and disease biomarkers in propionic acidemia (PA) and methylmalonic acidemia (MMA) patient hepatocytes cultured under hemodynamics and flow. A. 12C-propionyl-CoA (P-CoA) is greatly elevated in the PA and MMA derived donor cells compared to normal donor cells. B. Schematic of P-CoA pathways and derived metabolites. C. Propionylcarnitine to acetylcarnitine ratio (C3/C2 ratio) is greatly increased in the PA and MMA derived donor cells. D. Methylcitric acid (MCA), which is derived from P-CoA and oxaloacetate, is elevated in the PA and MMA cells compared to controls. E. Intracellular methylmalonyl-CoA levels are elevated in MMA patients. F. 13C-methylmalonic acid (13C-MMA) is produced from 13C-propiogenic sources in MMA patients. Error bars represent the 95% confidence interval of the mean estimate. All data is shown. There are 2 technical replicates per biological replicate. Biological replicates range from 2 to 11 depending on the donor and measurement.