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Fig. 2 | Molecular Brain

Fig. 2

From: A rare CACNA1H variant associated with amyotrophic lateral sclerosis causes complete loss of Cav3.2 T-type channel activity

Fig. 2

Electrophysiological characterization of Cav3.2 P1210L and ΔI153 variants. a Representative T-type current traces recorded in response to 150 ms depolarizing steps to values ranging between − 90 mV and + 30 mV from a holding potential of − 100 mV for wild-type (WT, black traces), P1210L (blue traces), and ΔI153 (red traces) channel variants expressed in tsA-201 cells. b Corresponding mean current-voltage relationship (I/V) for WT (black circles), P1210L (blue circles), and ΔI153 (red circles) channels. c Corresponding mean maximal macroscopic conductance (Gmax) obtained from the fit of the I/V curves with the modified Boltzmann eq. (1). d Mean normalized voltage-dependence of activation and inactivation for WT (black circles) and P1210L channels (blue circles). e Mean normalized recovery from inactivation kinetics. f Representative T-type current traces recorded from WT (black trace) and ΔI153 DRG neurons (red trace) 3 days after editing of Cacna1h by CRISPR/Cas9 in response to 80 ms depolarizing steps to − 25 mV from a holding potential of − 90 mV. g Corresponding mean peak T-type current density at − 25 mV in WT and ΔI153 mutant DRG neurons

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