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

Figure 2. An SMN-resistant state is acquired during adult life.

Figure 2

(A) Western blot analysis of SMN in tissues of inducible mice demonstrates dramatic depletion of protein 5 days following TM administration. Quantification of protein levels in (B) spinal cord and (C) skeletal muscle of treated mutants at the various time points examined. n ≥ 3 mice analyzed for each time point. (D) Loss of body weight and (E) early mortality of SMN-depleted mutants harboring 0 or 1 but not 2 SMN2 copies. (F) A delay in the onset of disease in mice bearing 1 versus 0 copies of the SMN2 gene. ***P < 0.001, t test, n ≥ 10 mice. (G) Representative image of 1- and 2-copy SMN2 inducible mutants 30 days following TM administration depict an alert, relatively healthy SMN2+/+ mouse compared with its hunched and somewhat disheveled looking hemizygous (SMN2+/–) littermate. (H) Two- but not one SMN2 copy in SMN-inducible mice protects against loss of forelimb strength. ***P < 0.001, t test, n ≥ 12 mice of each genotype. (I) Western blot depicting robust and equivalent depletion of SMN protein in the various cohorts of inducible mutants.