Fig. 4. Sephin1 prevents motor deficits, motor neuron loss, and the molecular defects in SOD1^mutant mice.

(A) Total body weight gain of wild-type or SOD1^mutant mice treated orally with Sephin1 (5 mg/kg) or vehicle once a day from 4 to 11 weeks of age. Data are means ± SEM (n = 4 to 6 mice). (B) Rotarod analysis of 110-day-old wild-type or SOD1^mutant mice treated as in (A). Data are means ± SEM (n = 4 to 6 mice). (C and D) Representative motor neuron staining (NeuN, green) and quantification (D) of sections of anterior horn of the lumbar region of spinal cord of 110-day-old mice treated as in (A). Nuclei, H33258 (blue). (D) Data are means ± SEM (n = 6 to 8 mice). (E) SOD1 immunoblots on soluble and insoluble fractions of spinal cord extracts from SOD1^mutant mice treated as in (A). (F) mRNA levels (quantitative PCR) in lumbar spinal cord from 4-month-old mice of indicated genotype, after treatment with Sephin1 or vehicle as in (A). Data are means ± SEM (n = 4 to 6 mice). *P ≤ 0.05; **P ≤ 0.01; ***P ≤ 0.001. Scale bar, 50 μm.