FIGURE 4.

Mildronate treatment eliminates acylcarnitines and improves lung function in LCAD^−/− mice. LCAD^−/− mice were treated with 200 mg/kg/day mildronate to induce carnitine deficiency. A, free carnitine concentration in mouse lung tissue (n = 4–5). B, changes in key species of long-chain acylcarnitines in the epithelial lining fluid. C, mildronate improved the levels of large aggregate surfactant phospholipids in LCAD^−/− lavage fluids (n = 9–10). D, mildronate did not improve the tissue elastance (H) parameter of lung function as determined with a FlexiVent small animal ventilator, but (E) did normalize pulmonary compliance determined by analysis of pressure-volume loops (n = 9–10). *, p < 0.01; **, p < 0.001; NS, not significant between indicated groups. All bars depict means and standard deviations.