Dysfunctional Mitochondria Accumulate in a Skeletal Muscle Knockout Model of Smn1, the Causal Gene of Spinal Muscular Atrophy

Scientists showed that survival motor neuron (SMN) loss in mouse skeletal muscle led to the accumulation of dysfunctional mitochondria. They tail vein injected GFP + AFS cells and first evaluated mitochondrial ultrastructure in TA muscle.