Altered postnatal maturation of electrical properties in spinal motoneurons in a mouse model of amyotrophic lateral sclerosis

Non‐technical summary  Our focus was on whether amyotrophic lateral sclerosis (ALS) might be precipitated by early developmental changes in large spinal motoneurons, which are vulnerable to early die‐off in ALS. It has been shown that some electrical properties in motoneurons are profoundly altered soon after birth in mutant superoxide dismutase‐1 (SOD1) mice, a standard animal model of ALS. These same properties undergo rapid developmental changes in normal mice during this time period. Our goal was to compare the development of motoneuron electrical properties in normal and SOD1 mice. Properties were measured from birth to 12 days of age, when the mouse is considered juvenile, but long before symptom onset. Most electrical properties in the SOD1 motoneurons showed an accelerated pace of maturation during this early developmental period compared with the normal motoneurons. If this trend persists, it could, along with other disease factors, hasten the onset of normal motoneuron degeneration due to ageing and result in the development of ALS.

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