Key result
The alpha-MHC403/+ mutation in mice resulted in more morphologically abnormal myocytes (49% vs 12% type III cells, P<0.01) and delayed intracellular Ca2+ signal decay (217 vs 159 ms, P<0.01).
Population
Isolated left ventricular myocytes from 15-week-old male mice bearing the Arg403 --> Gln alpha-cardiac…
Comparison
Arg403 --> Gln alpha-cardiac myosin heavy chain… vs Wild-type (WT) control myocytes
Design
Preclinical
Authors
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Should not change clinical practice in hypertrophic cardiomyopathy; leaves open whether direct myocyte effects drive human disease progression.
p-value: p=<0.01
The Arg403 --> Gln alpha-cardiac myosin heavy chain mutation directly impairs myocyte contraction and relaxation function, independent of secondary changes in global cardiac function or loading conditions.
Kim et al. (1999) studied Familial hypertrophic cardiomyopathy. Arg403 --> Gln alpha-cardiac myosin heavy chain missense mutation (alpha-MHC403/+) vs. Wild-type (WT) control cells was evaluated on Myocyte morphology and intracellular Ca2+ signal decay (p=<0.01). The alpha-MHC403/+ mutation in mice resulted in more morphologically abnormal myocytes (49% vs 12% type III cells, P<0.01) and delayed intracellular Ca2+ signal decay (217 vs 159 ms, P<0.01).
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