Extended Data Fig. 7: Related to Fig. 3: electrophysiologic characterization of Purkinje cells from Hpdl+/+, Hpdl+/−, Hpdl−/− and 4-HMA-treated Hpdl−/− mice.
From: Coenzyme Q headgroup intermediates can ameliorate a mitochondrial encephalopathy
a, The I/V curves of Purkinje cells from P8-10 Hpdl−/− pups and 4-HMA-treated Hpdl−/− pups are significantly different from each other and from the I/V curves of Hpdl+/+ and Hpdl+/− pups, which are similar. P-values indicating statistical significance are listed. The Hpdl−/− pups have a flatter I/V curve at hyperpolarized membrane potentials relative to the other curves due to a tighter grouping of currents at hyperpolarized membrane potentials (-100 to -60 mV) than the Hpdl+/+ and Hpdl+/− pups (see insert). The 4-HMA-treated Hpdl−/− pups have a steeper I/V curve similar to more mature Purkinje cells. b, The I/V curves of Purkinje cells from P17-19 Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− pups are similar. The inset depicts an example Hpdl+/+ Purkinje cell current response to the voltage step commands, which is similar to the Hpdl+/− and 4-HMA-treated Hpdl−/− pup Purkinje cells responses. c, The I/V curves of Purkinje cells from P56-57 Hpdl+/+, Hpdl+/−, and 4-HMA-supplemented Hpdl−/− mice are comparable. d, Peak frequency of Purkinje cells from P8-10 Hpdl+/+, Hpdl+/−, Hpdl−/− and 4-HMA-treated Hpdl−/− pups. The 4-HMA treated Hpdl−/− group has higher peak frequency than any of the other groups. Differences in frequency were identified by two-way ANOVA followed by Dunnett’s multiple comparison test. e, Peak frequency of Purkinje cells from P17-19 Hpdl+/+, Hpdl+/−, and 4-HMA-treated Hpdl−/− pups. The peak frequencies in all three groups are comparable. f, F/I curves of Purkinje cells from P8-10 Hpdl+/+, Hpdl+/−, Hpdl−/− and 4-HMA-treated Hpdl−/− pups. There were no significant differences in the linear portions of each curve between each group. The insert shows the simple firing pattern of an immature Purkinje cell. g, F/I curves of Purkinje cells from P17-19 Hpdl+/+, Hpdl+/−, Hpdl−/− and 4-HMA-treated Hpdl−/− pups. There were no significant differences in the linear portions of each curve between each group. The insert shows the simple firing pattern of a more mature Purkinje cell. h, Input resistance and membrane capacitance in Purkinje cells from P56-57 (8-week-old; age comparable to behavioral assays) Hpdl+/+ and 4-HMA-supplemented Hpdl−/− mice are comparable, with a nonsignificant decrease in input resistance and a nonsignificant increase in membrane capacitance in Hpdl+/− mice. i, The F/I curve of Purkinje cells from P56-57 Hpdl+/+ mice exhibits a higher firing rate at higher input currents than Purkinje cells from Hpdl+/− and 4-HMA-treated Hpdl−/− mice. All electrophysiological data are presented as the averages of at least 10 Purkinje cells (Supplementary Table 4). Significance was tested by two-way ANOVA followed by Tukey’s post-hoc test.
