Key result
Muscle fibres carrying the E41K beta-Tm mutation showed decreased speed of contraction and Ca(2+) sensitivity compared to controls, with Ca(2+) sensitivity being reversible by EMD 57033.
Population
Skinned muscle fibres from 2 patients carrying a novel E41K beta-tropomyosin mutation associated with…
Comparison
Exposure to 30 µm of the Ca sensitizer EMD 57033 vs Fibres from healthy controls and untreated…
Design
Preclinical
Authors
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EMD 57033 may restore Ca(2+) sensitivity in E41K fibers; leaves open therapeutic potential pending clinical studies.
Case-Control (n=10)
The E41K beta-Tm mutation impairs muscle contractile function by decreasing contraction speed and Ca(2+) sensitivity, but the latter can be improved with the Ca(2+) sensitizer EMD 57033, suggesting a potential therapeutic approach.
Ochala et al. (2008) conducted a case-control in Congenital myopathy and muscle weakness (n=10). E41K beta-Tm mutation vs. Healthy controls was evaluated on Speed of contraction and contraction sensitivity to Ca(2+) concentration. Muscle fibres carrying the E41K beta-Tm mutation showed decreased speed of contraction and Ca(2+) sensitivity compared to controls, with Ca(2+) sensitivity being reversible by EMD 57033.
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