Extended Data Fig. 4: Taz−/YAbhd18−/− mice are fertile and demonstrate normal heart function.
From: Genetic suppression features ABHD18 as a Barth syndrome therapeutic target
(a) Representative CL spectrum of control wildtype mice (n = 3 animals, left), and Abhd18 knockout mice (n = 3 animals, right) obtained by blood spot assay. Blue: summation of carbon atoms in the fatty acid side chains. Red: CL peaks for each carbon group along with number of double bonds. (b) qRT-PCR analysis of Taz and Abhd18 expression levels in mice heart tissue. Expression was normalized to Gapdh. Data are presented as means ± s.e.m. from three animals. (c) Schematic showing sterility test cross between Taz−/Y:Abhd18−/− double KO male mice and Taz+/−Abhd18−/− female mice. (d) Representative ultrasound images of mouse hearts recorded in M mode (WT n = 8, Taz−/Y n = 3, Abhd18−/− n = 7, Abhd18−/−Taz−/Y n = 6 mice). Six measurements were taken for each image using ImageJ: 1. Anterior Wall Thicknesss (AWT) – diastole (d), 2. Left Ventricle End Diastole Diameter (LVEDD), 3. Posterior Wall Thickness (PWT) – diastole (d), 4. AWT – systole (s), 5. Left Ventricle End Systole Diameter (LVESD) and 6. PWT – systole (s).
