Nerve Signals Contribute to Motor Neuron Death in Amyotropic Lateral Sclerosis

ALS, often referred to as "Lou Gehrig's disease", results in progressive loss of muscle control due to the death of motor neurons, which connect the brain and spinal cord with muscles. There is no current cure or treatment for ALS.

One potential cause of motor neuron death in ALS is hyperexcitability, which occurs when neurons are overactivated. Chang et al proposed that this hyperexcitability could be due in part to loss of inhibition of the neurons. They found that the number of a certain type of inhibitory neurons, glycinergic neurons, was reduced in a mouse model of ALS. Importantly, these neurons were lost before structural evidence of motor neuron degeneration was observed, suggestive a potential causative role.

Drs. Chang and Martin conclude that "selective loss of inhibitory glycinergic interneuron regulation of motoneuron function or glycinergic interneuron degeneration could contribute to motoneuron degeneration in ALS. It remains to be determined if interneuronal loss is due to pathology intrinsic to the interneuron or if it is secondary to very early primary disease in their target motoneurons."

By American Journal of Pathology