Pathophysiology of ion channels in amyotrophic lateral sclerosis

Stringer, Robin N.; Weiss, Norbert

doi:10.1186/s13041-023-01070-6

Molecular Brain

Table 1 Alterations of ion channels in ALS patients and preclinical rodent models

From: Pathophysiology of ion channels in amyotrophic lateral sclerosis

	Channel	Effect	Model	References
Hyperexcitability	Na_v1.2	Increase in persistent sodium current	Application of PR₂₀ on HEK-293 T cells expressing recombinant channel	[80]
	Na_v1.3	Hyperpolarizing shift in voltage dependence of activation	Spinal motor neurons from SOD1^A4V mice	[76]
	Na_v1.4	Reduction in mRNA expression	Skeletal muscle cells from the Tibialis Anterior muscle from SOD1^G93A mice	[93]
	Na_v1.6	Increase in protein expression	AIS of spinal motor neurons from SOD1^G127X 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 SOD1^G93A rats	[98]
	Kir4.1	Reduction in expression and activity	Oligodendrocytes of the ventral horns of SOD1^G93A rat lumbar and cervical spinal cords and myelin fraction from the spinal cord	[100]
	NCX3	Reduction in expression and activity	Spinal motor neurons from SOD1^G93A mice	[106, 108]
	Ca_v2.2	Increase in mRNA expression	Spinal motor neurons from SOD1^G93A mice	[112]
	Ca_v1.4	Increase in persistent current	Spinal motor neurons from SOD1^G93A mice	[112]
	CIC-1	Reduction in mRNA expression	Skeletal muscle from SOD1^G93A mice	[93]
Hypoexcitability	Na/K-ATPase	Reduction in protein expression	Spinal cord and cerebellum from SOD1^G93A mice	[36]
	Ca_V3.2	V1689M mutation—Depolarizing shift of the voltage dependence of activation	tsA-201 cells expressing recombinant channel	[132]
	Ca_V3.2	A1705T mutation—Hyperpolarizing shift of the voltage dependence of steady state inactivation	tsA-201 cells expressing recombinant channel	[132]
	Ca_v3.2	ΔI153 mutation—total loss of channel expression	tsA-201 cells expressing recombinant channel	[133]
	Ca_v3.2	P1210L mutation—decreased T-type current and channel expression	tsA-201 cells expressing recombinant channel	[133]

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ISSN: 1756-6606

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