Fig. 1

Effect of anserine on antioxidative capacity and cell viability in mut⁰ and control cells. (A) The presence of the genetic defect in immortalized kidney tubule epithelial cells (iKTEC) from three patients with clinically and molecular biologically confirmed loss of activity of methylmalonyl-CoA mutase (MMUT) was confirmed at protein level (by Western blot) before performing the experiments. C1-3 = Control patients; M1-3 = patients with mut⁰ defect. The figure is displayed as cropped version of the performed western blot, full-length gel is included in the supplementary information (Suppl. Fig. 1). (B) Antioxidative capacity, measured by ORAC, was reduced in iKTEC from 3 patients with methylmalonyl-CoA mutase deficiency (mut⁰) compared to immortalized cells from healthy individuals (Ctrl). Incubation with anserine (A, 2 mM) for 7 days increased the antioxidative capacity in cells from mut⁰ patients. (C) Cell viability (MTT assay) of iKTEC from patients was reduced in iKTEC from 3 mut⁰ patients. Incubation with anserine (2 mM) for 96 h had no influence on cell viability in cells from patients and controls. Statistical analysis was performed using a simple t-test for A and ordinary two-way ANOVA with Šídák’s multiple comparisons test for B and C.* = p < 0.05; ** = p < 0.01; **** = p < 0.0001. There is no significance between the groups, except for the ones indicated in the graphs. Antioxidant capacity and viability are shown as percentages relative to control cells in normal medium (dashed line = 100%). Symbols/lines in the violin plots represent the mean value for one patient analyzed in three independent experiments, each performed with 5–9 technical replicates.