Fig. 2: Lack of dysferlin synergistically increases RyR1 leak via DHPR destabilization and RyR1 phosphorylation. | Nature Communications

Fig. 2: Lack of dysferlin synergistically increases RyR1 leak via DHPR destabilization and RyR1 phosphorylation.

From: Muscle-specific Ryanodine receptor 1 properties underlie limb-girdle muscular dystrophy 2B/R2 progression

Fig. 2: Lack of dysferlin synergistically increases RyR1 leak via DHPR destabilization and RyR1 phosphorylation.The alternative text for this image may have been generated using AI.

a, b Representative [Ca2+]t-sys(t) in WT (a) and BlaJ (b) psoas fibre during sequential perfusion of nifedipine (5 mM) and tetracaine (1 mM). c Percentage of nifedipine-sensitive (black) and nifedipine-insensitive RyR1 leak WT and BlaJ psoas fibres. n = 5–8. d T-system rhod-5N confocal image of two TA fibres placed in parallel to each other for simultaneous live imaging. Scale bar: 100 µm. e Representative simultaneous [Ca2+]t-sys traces from WT and BlaJ TA fibres exposed to cAMP (100 mM) and tetracaine (1 mM) subsequently. f Tetracaine-induced Δ[Ca2+]t-sys of cAMP-exposed WT and BlaJ TA fibres. Summary of results shown in (e). n = 7. a–f 8 weeks-old fibres. Statistical analysis in (c), unpaired t-test. Statistical analysis in (f), paired t-test. **p < 0.01. Source data are provided as a Source Data file.

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