Key result
Heterozygous cMyBP-C null mice develop asymmetric septal hypertrophy and fibrosis at 10-11 months of age, representing a key phenotypic feature of human familial hypertrophic cardiomyopathy.
Population
cMyBP-C null mice (homozygotes and heterozygotes) generated by targeted deletion of exons 1 and 2
Design
Preclinical
Follow-up
up to 10-11 months of age
Authors
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Establishes first cMyBP-C mouse model with asymmetric septal hypertrophy; extends prior alleles but remains hypothesis-generating for human translation.
Heterozygous cMyBP-C null mice represent the first model to exhibit asymmetric septal hypertrophy, a key feature of human familial hypertrophic cardiomyopathy.
L. Carrier (2004) studied Familial hypertrophic cardiomyopathy. cMyBP-C null mutation (homozygous and heterozygous) was evaluated on Cardiac phenotype (hypertrophy, fractional shortening, relaxation, fibrosis). Heterozygous cMyBP-C null mice develop asymmetric septal hypertrophy and fibrosis at 10-11 months of age, representing a key phenotypic feature of human familial hypertrophic cardiomyopathy.
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