Extended Data Fig. 9: Related to Fig. 3: additional metabolic, behavioral, and neurological tests of Hpdl+/+, Hpdl+/−, Hpdl−/− and 4-HMA-treated Hpdl−/− mice.
From: Coenzyme Q headgroup intermediates can ameliorate a mitochondrial encephalopathy
a, Metabolic phenotypes such as food and water intake, activity, O2 consumption, CO2 production, and energy expenditure are similar in 4-6-week-old Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− mice, as measured in metabolic cages. There were no significant changes in any of these parameters between Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− mice. b, Respiratory exchange ratio (vO2/vCO2) in Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− mice. c, 4-HMA treatment partially restores the weight of male Hpdl−/− mice and rescues the weight of Hpdl−/− female mice. Hpdl+/− mice have comparable weights to Hpdl+/+ mice. The combined data demonstrate that 4-HMA partially restores the weight of Hpdl−/− mice relative to Hpdl+/− mice. d, Respiratory exchange ratio summary in Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− mice. Male Hpdl−/− mice treated with 4-HMA had a significantly increased respiratory exchange ratio (RER) compared to both Hpdl+/+ male mice and Hpdl+/− and 4-HMA-treated Hpdl−/− female mice. e, Rotarod performance is similar in Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− male and female mice. f, Open field data from 6–8-week-old Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− mice revealed no significant differences in distance moved or time that mice spent in the center of the open field. For behavioral tests, data from male mice (n: Hpdl+/+= 4, Hpdl+/−= 5, 4-HMA-treated Hpdl−/−=4) are plotted as triangles. Data from female mice (n: Hpdl+/+= 4, Hpdl+/−=2, 4-HMA-treated Hpdl−/−=5) are plotted as circles. Behavioral data do not contain comparisons to untreated Hpdl−/− mice, which do not survive to the age at which these behavioral assays are carried out. Data presented are mean ± standard error of the mean, as well as raw data. Behavioral data are presented as box and whisker plots, where the center is the median, the box bounds are the 25th to 75th percentiles, and whiskers indicate minimum to maximum. Significance was tested by two-way ANOVA followed by Tukey’s post-hoc test.
