From: Pathophysiology of ion channels in amyotrophic lateral sclerosis
 | Channel | Effect | Model | References |
---|---|---|---|---|
Hyperexcitability | Nav1.2 | Increase in persistent sodium current | Application of PR20 on HEK-293Â T cells expressing recombinant channel | [80] |
Nav1.3 | Hyperpolarizing shift in voltage dependence of activation | Spinal motor neurons from SOD1A4V mice | [76] | |
Nav1.4 | Reduction in mRNA expression | Skeletal muscle cells from the Tibialis Anterior muscle from SOD1G93A mice | [93] | |
Nav1.6 | Increase in protein expression | AIS of spinal motor neurons from SOD1G127X mice | [79] | |
KCNQ2 | Reduction in mRNA expression | Spinal motor neurons of ALS patients | [96] | |
KCNA1 | Reduction in mRNA expression | Spinal motor neurons of ALS patients | [96] | |
KCNA2 | Reduction in mRNA expression | Spinal motor neurons of ALS patients | [96] | |
Kir4.1 | Reduction in expression and activity | Cortex and brainstem astrocytes of SOD1G93A rats | [98] | |
Kir4.1 | Reduction in expression and activity | Oligodendrocytes of the ventral horns of SOD1G93A rat lumbar and cervical spinal cords and myelin fraction from the spinal cord | [100] | |
NCX3 | Reduction in expression and activity | Spinal motor neurons from SOD1G93A mice | ||
Cav2.2 | Increase in mRNA expression | Spinal motor neurons from SOD1G93A mice | [112] | |
Cav1.4 | Increase in persistent current | Spinal motor neurons from SOD1G93A mice | [112] | |
CIC-1 | Reduction in mRNA expression | Skeletal muscle from SOD1G93A mice | [93] | |
Hypoexcitability | Na/K-ATPase | Reduction in protein expression | Spinal cord and cerebellum from SOD1G93A mice | [36] |
CaV3.2 | V1689M mutation—Depolarizing shift of the voltage dependence of activation | tsA-201 cells expressing recombinant channel | [132] | |
CaV3.2 | A1705T mutation—Hyperpolarizing shift of the voltage dependence of steady state inactivation | tsA-201 cells expressing recombinant channel | [132] | |
Cav3.2 | ΔI153 mutation—total loss of channel expression | tsA-201 cells expressing recombinant channel | [133] | |
Cav3.2 | P1210L mutation—decreased T-type current and channel expression | tsA-201 cells expressing recombinant channel | [133] |