Table 1.

Alteration of GSH content in various experimental models of ALS.

Cell Lines and Animals	Experimental ALS Models	Phenotypes Associated with Oxidative Stress	GSH Status	Reference
NT-2 SK-N-MC	Human SOD1WT Human SOD1G37R Human SOD1G85R	Increased protein carbonyl Increased 8-OHG Increased lipid peroxidation	Decrease GSH Increased GSSG	[69]
NSC-34	Ethacrynic acid treatment	Decreased mitochondrial membrane potential Increased ROS generation Increased apoptosis	Decreased GSH	[70]
NSC-34	Human SOD1WT Human SOD1G93A	Decreased cell viability Increased ROS generation Decreased mitochondrial membrane potential Increased mitochondrial toxicity	-	[71]
NSC-34	Ethacrynic acid treatment	Increased ROS generation Increased oxidative response gene expression Increased apoptosis	Decreased GSH	[72]
NSC-34	Human SOD1G93A	Decreased cell proliferation Decreased cell viability Increased apoptosis	Decreased mitochondrial GSH	[73]
NSC-34	Human SOD1WT Human SOD1G93A	Increased mitochondrial dysfunction	Decreased GSH	[76]
NSC-34	Human TDP-43M337V	Increased gene expression of Nrf2 signaling pathway	Decreased GSH	[78]
NSC-34	Human TDP-43WT Human TDP-43A315T	Increased ROS generation Decreased cell viability Increased cell death	Decreased GSH	[79]
NSC-34	Human SOD1G93A	Restored cell viability by treating urate	Increased GSH by treating urate	[98]
Rat astrocytes	Human SOD1G93A	Restored cell survival by activating Nrf2	Increased GSH by activating Nrf2	[85]
Mouse motor neurons	Human SOD1G93A	Increased apoptosis	Decrease GSH Increased GSSG	[72]
Mouse astrocytes	Human SOD1G93A	Extended survival by expressing Nrf2 Delayed muscle denervation by expressing Nrf2	Increased GSH secretion by expressing Nrf2	[86]
Mouse astrocytes	Human SOD1G93A Human SOD1H46R/H48Q	Decreased cell survival by GCLM knockout Increased motor neuron loss by GCLM knockout Increased oxidative stress by GCLM knockout Decreased complex IV activity by GCLM knockout	Decrease GSH by GCLM knockout	[88]
Mouse	Human SOD1G93A	Increased cystine uptake by cystine/glutamate antiporter	-	[94]
Fly	Human SOD1WT Human SOD1G85R	Extended survival by treatment with urate Improved motor defect by treatment with urate Enhanced antioxidant enzyme activity by treatment with urate Decreased ROS level by treatment with urate	-	[98]
Mouse	Human TDP-25	Increased memory deficit by treatment with dexamethasone	Decreased GSH/GSSG ratio by treatment with dexamethasone	[103]
Mouse	Human SOD1G93A	Decreased survival	Decreased GSH in whole blood and spinal cord	[87]
Mouse	Human SOD1WT	Decreased cell survival by GCLM knockout Increased motor neuron loss by GCLM knockout	Decrease GSH by GCLM knockout	[89]