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. 2014 Jan 27;124(2):785–800. doi: 10.1172/JCI72017

Figure 7. An SMN refractory state emerges concomitant with the establishment of the fully mature neuromuscular synapse.

Figure 7

(A) Kaplan-Meier curves indicate a dramatic improvement in survival if SMN depletion is effected in mice of P15 or greater. (B) Immunostains of motor neurons from P12 and P15 TM-treated mice indicate equivalent low levels of SMN, confirming the efficiency of SMN depletion at the 2 ages. Scale bar: 30 μm. (C) Quantification of nuclear gems in motor neurons of the 2 sets of mice did not reveal a difference, confirming equally effective SMN depletion in the 2 cohorts. n ≥ 400 motor neuron nuclei from n = 3 mice of each genotype examined, 1-way ANOVA. (D) Analysis of body weights of P15, P21, and P50 TM-treated 2 SMN2 copy SmnF7/– mice with or without CreER indicates small but significant improvement in the phenotypes of mutants depleted of SMN later than P15. *P < 0.05; **P < 0.01. n ≥ 4 mice of each genotype, t test.