Population
ACTC/KO mouse model and age-matched wild-types
Comparison
Transgenic over-expression of cardiac α-actin in… vs Age-matched wild-type mice
Design
Preclinical
Key result
Transgenic over-expression of cardiac α-actin in ACTC(Co)/KO mice resulted in 20 to 25% less myofibre force production and lower stiffness compared with age-matched wild-types.
Authors
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Supports isoform effects on myofibre mechanics in mice; leaves open relevance to ACTA1 gene therapy design.
The presence of cardiac α-actin instead of skeletal muscle α-actin alters actin conformational changes and lowers myofibre force production, which has implications for designing gene therapies for ACTA1-based congenital myopathies.
Ochala et al. (2013) studied Congenital myopathies (ACTA1 null mutations). Transgenic over-expression of cardiac α-actin vs. Age-matched wild-types was evaluated on Myofibre force production and stiffness. Transgenic over-expression of cardiac α-actin in ACTC(Co)/KO mice resulted in 20 to 25% less myofibre force production and lower stiffness compared with age-matched wild-types.