Figure 1.
Sfpq Mutants Exhibit Skeletal Muscle Growth Defects with Kyphosis and Premature Death
(A) Growth charts of male skeletal muscle-specific Sfpq homozygous knockout mice (KO-homo) (n = 4), heterozygous knockout mice (KO-hetero) (n = 7), and littermate control mice (n = 16). Data are presented as mean ± SD. *p < 0.05, **p < 0.01, ***p < 0.001 versus control (Student's or Welch's t test).
(B) Kaplan-Meier survival curves for male and female KO-homo (n = 14) and control (KO-hetero and others) (n = 27) mice.
(C) Kyphosis phenotype observed in P35 male KO mice.
(D) Body weight and weight of various skeletal muscles in male KO and control mice (n = 5). Representative skeletal muscle samples from KO and control mice (right). DIA, diaphragm; GC, gastrocnemius; SOL, soleus; TA, tibialis anterior. Data are presented as mean ± SD. ***p < 0.001 (Student's test).
(E) Grip strength test performance normalized to body weight of male KO (n = 19) and control (n = 20) mice from P26 to P30. Data are presented as mean ± SEM. ***p < 0.001 (Student's t test).
(F) Cross-section of laminin-stained TA muscle from 1-month-old male KO and control mice (left). Scale bars: 100 μm. Histogram of cross-sectional area (μm2) of laminin-stained myofibers from KO and control mice (right) (n = 3). Data are presented as mean ± SD. The difference of the distribution of cross-sectional area between KO and control mice was statistically significant (p < 0.001) (totally nine sections from three mice in each genotypes) using Mann-Whitney U test.
(G) Number of myofibers in TA muscle from 1-month-old male KO and control mice (n = 3, totally three sections from three mice in each genotype). Data are presented as mean ± SEM. There was no significant difference with Student's t test.
See also Figure S1.