Comparison of Two Murine Models of Familial Hypertrophic Cardiomyopathy
Population
Heterozygous mice bearing a cardiac MHC missense mutation (alphaMHC) or a cardiac MyBP-C mutation (MyBP-C)…
Comparison
alphaMHC(403/+) mutation model vs MyBP-C(t/+) mutation model
Design
Preclinical
Follow-up
up to >125 weeks
Authors
Discussion
Loading...
Member takes
Implication
Supports mutation-specific HCM modeling in mice; leaves open translation to human risk stratification or therapy.
Structured PICO
P
Population
Heterozygous mice bearing a cardiac MHC missense mutation (alphaMHC(403/+)) or a cardiac MyBP-C mutation (MyBP-C(t/+)) as murine models of familial hypertrophic cardiomyopathy (FHC)
I
Intervention
alphaMHC(403/+) mutation model
C
Comparator
MyBP-C(t/+) mutation model
O
Outcome
Cardiac structure and function (including left ventricular hypertrophy, molecular markers of hypertrophy, inducible ventricular tachycardia, and cardiac function impairment)surrogate
Murine models of familial hypertrophic cardiomyopathy accurately mimic the clinical severity of human mutations, with MHC mutations causing more severe structural, functional, and electrophysiological impairment than MyBP-C mutations.
Cite This Study
McConnell et al. (2001) studied this question.