To assess the role of CPT1A in the development and progression of ALS, we generated a SOD1G93A mouse model with a heterozygote Cpt1a P479L mutation (SOD1G93A/Cpt1a) with 66% CPT1A activity compared to the wildtype CPT1A (Fig. 4f). The gene discussed is CPT1A; the disease is amyotrophic lateral sclerosis.