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. 2013 Oct 8;5(12):1887–1900. doi: 10.1002/emmm.201303275

Figure 6.

Enhanced muscle pathology in Mbnl1; Mbnl2 muscle-specific KO mice.

  • A. Images of left hindlimb (left large panels), and dissected quadripceps muscles (small right panels), of WT (Mbnl1+/+; Mbnl2+/+; Myo-Cre+/−), Mbnl1 KO (Mbnl1−/−; Mbnl2c/c; Myo-Cre−/−) and Mbnl1−/−; Mbnl2c/c; Myo-Cre+/− DKOs.

  • B. H&E staining of transverse muscle sections in tibialis anterior (TA) muscles of WT (Mbnl1+/+; Mbnl2+/+; Myo-Cre+/−), Mbnl1 KO (Mbnl1−/−; Mbnl2c/c; Myo-Cre−/−) and Mbnl1−/−; Mbnl2c/c; Myo-Cre+/− DKOs.

  • C. Increased centralized myonuclei in Mbnl1−/−; Mbnl2c/c; Myo-Cre+/− DKOs (purple) versus WT (green, Mbnl1+/+; Mbnl2+/+; Myo-Cre+/−), Mbnl1−/− KO (red, Mbnl1−/−; Mbnl2c/c; Myo-Cre−/−) and Mbnl1−/−; Mbnl2+/− (blue) mice (n = 2 per genotype, *p = 0.00208, **p = 9.3E-06, ***p = 2.7E-10, Student's t-test).

  • D. Decreased grip strength in Mbnl1−/−; Mbnl2c/c; Myo-Cre+/− DKOs (purple) versus WT (green), Mbnl1 KO (red) and Mbnl1−/−; Mbnl2+/− (blue) mice (15 weeks of age, n = 11 for WT and Mbnl1−/−, 5 for Mbnl1−/−; Mbnl2+/− and 2 for Myo-Cre+/− DKO; *p = 7.7E-06, **p = 0.03337, Student's t-test).

  • E. RT-PCR analysis showing profound increases in Bin1 exon 11 and Cacna1s exon 29 skipping when the expression of both Mbnl1 and Mbnl2 is eliminated in Mbnl1−/−; Mbnl2c/c; Myo-Cre+/− DKO (Myo-cre DKO) skeletal muscle.

  • F. Percent spliced-in (Ψ) values for five target pre-mRNAs in WT (green), Mbnl1−/− (red), Mbnl1−/−; Mbnl2+/− (blue) and Mbnl1−/−; Mbnl2c/c; Myo-Cre+/− DKO (Myo-Cre DKO) quadriceps muscles (n ≥ 2, *p = 0.0069 [Bin1, WT vs. Mbnl1−/−], 0.00047 [Bin1, Mbnl1−/− vs. Mbnl1−/−; Mbnl2+/−], 8.7E-06 [Bin1, Mbnl1−/−; Mbnl2+/− vs. Myo-Cre+/− DKO], 0.00078 [Cacna1s, WT vs. Mbnl1−/−], 0.00158 [Cacna1s, Mbnl1−/− vs. Mbnl1−/−; Mbnl2+/−], 2.1E-06 [Cacna1s, Mbnl1−/−; Mbnl2+/− vs. Myo-Cre+/− DKO], 0.00055 [Csda, WT vs. Mbnl1−/−], 0.00809 [Csda, Mbnl1−/− vs. Mbnl1−/−; Mbnl2+/−], 0.00108 [Csda, Mbnl1−/−; Mbnl2+/− vs. Myo-Cre+/− DKO], 7.2E-05 [Abi2, WT vs. Mbnl1−/−], 0.00876 [Abi2, Mbnl1−/−; Mbnl2+/− vs. Myo-Cre+/− DKO], 0.006 [Lnp, WT vs. Mbnl1−/−], 0.00825 [Lnp, Mbnl1−/−; Mbnl2+/− vs. Myo-Cre+/− DKO], Student's t-test).

Figure 6