Mouse Genome Informatics
ht
    Myh6tm1Jse/Myh6+
129S.129X1-Myh6tm1Jse
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Myocyte disarray and cardiac fibrosis in Myh6tm1Jse/Myh6+ mice

cardiovascular system
• myocyte disarray is seen in 29% of young and 40% of adult mutants, usually in the left ventricular wall or interventricular septum
• develop cardiac hypertrophy with increasing age such that by 20-30 weeks of age, hypertrophy is uniformly present
• mutants with inducible arrhythmias have greater left ventricular wall thickness and greater hypercontractility than mutants without inducible arrythmias, however observe no correlation between wall thickness and amount of fibrosis or myocyte disarray
• develop difusse and focal cardiac fibrosis with increasing age; total amount of fibrosis within each heart varies broadly
• end-diastolic left ventricle dimensions are smaller and fractional shortening is increased
• mutants with inducible arrhythmias have greater hypercontractility than mutants without inducible arrythmias
• ventricular tachyarrhythmia is induced in 25% of young and 69% of adults by rapid ventricular pacing at a cycle length of more than or equal to 50 ms; no arrhythmias are induced in wild-type

muscle
• end-diastolic left ventricle dimensions are smaller and fractional shortening is increased
• mutants with inducible arrhythmias have greater hypercontractility than mutants without inducible arrythmias

Mouse Models of Human Disease
OMIM IDRef(s)
Cardiomyopathy, Familial Hypertrophic, 14; CMH14 613251 J:104363