Population
Transgenic mice expressing varying amounts of a mutated MyBP-C or wild-type MyBP-C, and non-transgenic…
Comparison
Transgenic expression of truncated MyBP-C or… vs Non-transgenic littermates and mice expressing…
Design
Preclinical
Follow-up
up to 25 weeks (some observations up to 7 months)
Key result
Expression of a truncated MyBP-C lacking myosin and titin binding domains in transgenic mice led to sarcomere disorganization, increased Ca2+ sensitivity, and decreased maximum power output.
Authors
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Offers a mouse model for HCM sarcomere defects; leaves open translation to human disease mechanisms and therapies.
Expression of truncated MyBP-C in a transgenic mouse model leads to sarcomere disorganization, increased calcium sensitivity, and reduced power output, providing a mechanistic model for familial hypertrophic cardiomyopathy.
Yang et al. (1998) studied Familial hypertrophic cardiomyopathy (FHC). Transgenic expression of mutated MyBP-C (MyBP-C.mut1) vs. Nontransgenic littermates and wild-type MyBP-C transgenic mice was evaluated on Sarcomere organization and cardiac fiber mechanics (Ca2+ sensitivity and power output). Expression of a truncated MyBP-C lacking myosin and titin binding domains in transgenic mice led to sarcomere disorganization, increased Ca2+ sensitivity, and decreased maximum power output.