Mouse model for hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) caused by autosomal-dominant mutations in genes that code for the structural proteins of the sarcomere, is the most common inherited heart disease. HCM is associated with progressive myocardial hypertrophy and fibrosis, ventricular dysfunction, and arrhythmias. Disease onset during childhood and adolescence carries the risk of morbidity and sudden cardiac death. Hypoxia and the main regulator of the cellular hypoxic response hypoxia-inducible transcription factor-1a (HIF1A) have been associated with HCM, however their exact roles are not elucidated yet.