DEV010702 Supplementary Material

Files in this Data Supplement:

• Supplemental Figure S1 -

  Fig. S1. Mouse weight, muscle weight, muscle fiber diameter and AChR surface levels are normal in AchR-β^3F/3F mice. (A) Wild-type and AchR-β^3F/3F mice weigh similar amounts: wild-type, 24.9±4.3 g, n=3; AchR-β^3F/3F, 24.6±1.6 g, n=9; P>0.05, unpaired t-test. (B) Muscle weight (wet weight of diaphragm muscles) is normal in P0 AchR-β^3F/3F mice: wild-type, 7.3±0.5 mg, n=7; AchR-β^3F/3F, 7.4±0.6 mg, n=11; P>0.05, unpaired t-test). (C) Muscle fiber diameter is normal in P30 AchR-β^3F/3F mice. Sections (1 μm) from P30 diaphragm muscles, embedded in Epon, were stained with 0.1% Toludine Blue and images captured with a DKC500 camera (Sony, Tokyo, Japan); image analysis was performed with ImageJ 1.36. Wild-type, 100±7.7%, n=2; AchR-β^3F/3F, 127±29%, n=2; P>0.05, unpaired t-test. Scale bar: 100 μm. (D) Surface AChR expression is normal in AchR-β^3F/3F mice. Diaphragm muscles from P0 AchR-β^3F/3F mice and wild-type littermates were dissected in oxygenated L15 medium and labeled with ¹²⁵I-α-BGT. The bound radioactivity was measured in a gamma counter and normalized to the wet weight of the muscle: wild-type, 100±13.0%, n=7; AchR-β^3F/3F, 85.4±5.1%, n=11; P>0.05, unpaired t-test.