The goal of such treatments is to deliver alternate metabolic substrates that bypass the problem and may restore metabolic function in glucose-starved cells. To date, the only therapy offered to ALS patients to extend survival is riluzole, which offers only a modest extension of survival in some patients, and has considerable side effects. Therefore, studies on ALS SB 239063 inhibitor transgenic mice are crucial to test potential therapies that not only improve motor function, but extend survival, especially if anecdotal reports in humans suggest a therapeutic effect. Interestingly, although we observed an increase in survival for the SD+DP and the KD+DP groups, this effect has not been observed by others using therapies that target energy metabolism. These results support further research, since increased motor function adds to improved Delpazolid quality of life, and extension of survival time is a primary clinical goal for ALS patients. It is unclear whether the primary site of toxicity in the SOD1- G93A mice is in the skeletal muscle or the motor neurons, but it is likely that the mutated toxic form of SOD1 is expressed in more tissue types. Therefore, supporting the mitochondrial function of many cell types is a desirable therapeutic approach. Recent studies have shown that even a complete rescue of motor neuron cell bodies does not cure mSOD1 mice suggesting that preserving the normal function of motor neuron cells is therapeutically not sufficient, since the rescued motor neurons are unable to recreate destroyed neuromuscular junctions. Other attempts that rescue only motor neurons have also failed to halt progression. The primary site of mSOD1 toxicity is likely to be represented in several other cell types, such as glial cells and muscle fibers. Recent study shows that astrocytes expressing mSOD1 were able to trigger motor neuron death through a mechanism involving oxidative stress and NGF production. Other studies showed similar results suggesting that the astrocyte could be a site of mSOD1 toxicity. Indeed, decreasing mSOD1 expression in astrocytes also delayed disease onset in mSOD1 mice. Other studies highlight the importance of the interdependence between neurons and astroglial cells.