Extended Data Fig. 2: Related to Fig. 1: CoQ concentrations in the brains of pups and P90 mice following 4-HMA and 4-HB treatment.
From: Coenzyme Q headgroup intermediates can ameliorate a mitochondrial encephalopathy
a, CoQ9 and CoQ10 concentrations from mouse brains following treatment with 13C6-4-HMA or 13C6-4-HB starting at postnatal day 3. 13C6-4-HMA and 13C6-4-HB are incorporated into CoQ9 and CoQ10 in Hpdl−/− mice, and increase brain CoQ9 and CoQ10 pools relative to untreated Hpdl−/− mice. 13C6-4-HMA and 13C6-4-HB are minimally incorporated into CoQ9 and CoQ10 in Hpdl+/+ mice and are incorporated into CoQ9 and CoQ10 at low fractions in Hpdl+/− mice. 13C6-4-HMA: Hpdl+/+ n = 3, Hpdl+/− n = 6, and Hpdl−/− treated n = 5. 13C6-4-HB: Hpdl+/+ n = 3, Hpdl+/− n = 4, and Hpdl−/− treated n = 3. Hpdl−/− untreated n = 3. CoQ9 and CoQ10 data from Hpdl−/− untreated mice were obtained in the 4-HMA experiment and are presented in a faded shade of color in the 4-HB experiment for ease of interpretation. b, CoQ9 and CoQ10 concentrations from the cortex and cerebellum of mice treated with 13C6-4-HMA from P2-P30 and sacrificed at P90. The concentrations of CoQ9 and CoQ10 in the brains of treated Hpdl−/− mice are ~50% of the concentrations of these molecules in the brains of Hpdl+/+ mice. Only a minor fraction of CoQ9 and CoQ10 is labeled with 13C. Hpdl+/+ n = 3, Hpdl+/− n = 3, and Hpdl−/− treated n = 3. c, CoQ9 and CoQ10 concentrations from the brains of P90 wild-type mice that were not treated with 4-HMA, or treated with 4-HMA from P2-P30. 4-HMA does not increase the brain concentrations of CoQ9 and CoQ10 in wild-type mice. Significance was tested by two-way analysis of variance (ANOVA) followed by Tukey’s post-hoc test.
