scholarly journals Functional effects of troponin T mutations found in hypertrophic cardiomyopathy on the Ca2+-activated contraction of skinned cardiac muscle fibers

2000 ◽  
Vol 82 ◽  
pp. 116
Author(s):  
Sachio Morimoto ◽  
Keita Harada ◽  
Fumi Takahashi ◽  
Reiko Minakami ◽  
Iwao Ohtsuki
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Cardiac Muscle ◽  
Troponin T ◽  
Muscle Fibers ◽  
Cardiac Muscle Fibers

Ca2+-sensitizing effects of the mutations at Ile-79 and Arg-92 of troponin T in hypertrophic cardiomyopathy

1998 ◽  
Vol 275 (1) ◽  
pp. C200-C207 ◽  
Author(s):  
Sachio Morimoto ◽  
Fumi Yanaga ◽  
Reiko Minakami ◽  
Iwao Ohtsuki
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Cardiac Muscle ◽  
Contractile Function ◽  
Troponin T ◽  
Wild Type ◽  
Physiological Conditions ◽  
In The Wild ◽  
Tension Generation ◽  
Cardiac Muscle Fibers ◽  
Muscle Contractile

Several mutations in human cardiac troponin T (TnT) gene have been reported to cause hypertrophic cardiomyopathy (HCM). To explore the effects of the mutations on cardiac muscle contractile function under physiological conditions, human cardiac TnT mutants, Ile79Asn and Arg92Gln, as well as wild type, were expressed in Escherichia coli and exchanged into permeabilized rabbit cardiac muscle fibers, and Ca2+-activated force was determined. The free Ca2+ concentrations required for tension generation were found to be significantly lower in the mutant TnT-exchanged fibers than in the wild-type TnT-exchanged fibers, whereas no significant differences were found in tension-generating capability under maximal activating conditions and in cooperativity. These results suggest that a heightened Ca2+ sensitivity of cardiac muscle contraction is one of the factors to cause HCM associated with these TnT mutations.

scholarly journals Hypercontractile Properties of Cardiac Muscle Fibers in a Knock-in Mouse Model of Cardiac Myosin-binding Protein-C

2000 ◽  
Vol 276 (7) ◽  
pp. 5353-5359 ◽  
Author(s):  
Christian C. Witt ◽  
Brenda Gerull ◽  
Michael J. Davies ◽  
Thomas Centner ◽  
Wolfgang A. Linke ◽  
...  
Keyword(s):  
Mouse Model ◽  
Cardiac Muscle ◽  
Protein C ◽  
Binding Protein ◽  
Muscle Fibers ◽  
Cardiac Myosin ◽  
Myosin Binding Protein ◽  
Myosin Binding Protein C ◽  
Myosin Binding ◽  
Cardiac Muscle Fibers
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