Extended Data Fig. 6: Related to Fig. 3: electron flow measurements in cerebellum and cortex from Hpdl+/+, Hpdl+/−, Hpdl−/− and 4-HMA-treated Hpdl−/− mice.
From: Coenzyme Q headgroup intermediates can ameliorate a mitochondrial encephalopathy
a, Electron transport chain substrates and inhibitors used for the electron flow assay. I, Complex I. II, Complex II. III, Complex III. IV, Complex IV. Q, CoQ. b, Electron flow measurements of mitochondria isolated from the cortex of P9 Hpdl+/+, Hpdl+/−, and Hpdl−/− pups, as well as Hpdl−/− pups treated with 4-HMA. Electron flow through complex I and complex II is reduced in Hpdl−/− pups, and is improved by treatment with 4-HMA. c, Electron flow measurements of mitochondria isolated from the cortex and cerebellum of P90 (3-month-old) Hpdl+/+ and Hpdl+/− pups, as well as Hpdl−/− pups treated with 4-HMA from P3-30. Electron flow through complexes I, II, and IV are similar in all groups. d, Electron flow measurements of mitochondria isolated from P12 Hpdl+/+ mice treated with 4-HMA starting on P3. 4-HMA treatment does not increase electron flow through complex I, II, or IV in wild-type pups. Mitochondria were isolated from n = 5 animals for all groups. Significance was tested by two-way analysis of variance (ANOVA) followed by Tukey’s post-hoc test.
