@entity1121 ( @entity1121 ) is associated with @entity1283 , @entity67 , and @entity944 . Recently, it has been suggested that inefficient energy utilization could be a common molecular pathway of @entity1121 -related mutations. We have previously generated transgenic @entity35 overexpressing a truncated cardiac troponin T (DEL- @entity17509 ) molecule, displaying typical features of @entity1121 such as @entity67 and an increased susceptibility to @entity943 . We now studied these @entity35 using 31P magnetic resonance spectroscopy (MRS). MRS demonstrated that cardiac energy metabolism was markedly impaired, as indicated by a decreased @entity1734 to @entity855 ratio (-31%, p < 0.05). In addition, we assessed contractility of isolated cardiomyocytes. While DEL- @entity17509 and control cardiomyocytes showed no difference under baseline conditions, DEL- @entity17509 cardiomyocytes selectively exhibited a decrease in fractional shortening by 28% after 1 h in @entity413 -deprived medium (p < 0.05). Moreover, significant decreases in contraction velocity and relaxation velocity were observed. To identify the underlying molecular pathways, we performed transcriptional profiling using real-time PCR. DEL- @entity17509 hearts exhibited induction of several genes critical for cardiac energy supply, including @entity19182 , @entity70241 , and @entity6229 . Finally, DEL- @entity17509 @entity35 and controls were studied by radiotelemetry after being stressed by @entity3044 , revealing a significantly increased frequency of @entity943 in transgenic animals. In summary, we demonstrate profound energetic alterations in DEL- @entity17509 hearts, supporting the notion that inefficient cellular @entity855 utilization contributes to the pathogenesis of @entity1121 .

Decreased contractility due to energy deprivation in a transgenic XXXX model of @entity1121 .

@entity1734 :: ('CHEBI:17287', 'Chemical') :: ['phosphocreatine'] @entity19182 :: ('29184(Tax:10116)', 'Gene') :: ['CD36'] @entity943 :: ('MESH:D001145', 'Disease') :: ['ventricular arrhythmias', 'arrhythmias'] @entity944 :: ('MESH:D003645', 'Disease') :: ['sudden death'] @entity1283 :: ('MESH:D006332', 'Disease') :: ['cardiac hypertrophy'] @entity17509 :: ('24838(Tax:10116)', 'Gene') :: ['TNT'] @entity6229 :: ('83516(Tax:10116)', 'Gene') :: ['PGC-1alpha'] @entity70241 :: ('25413(Tax:10116)', 'Gene') :: ['CPT-1/-2'] @entity3044 :: ('MESH:D007545', 'Chemical') :: ['isoproterenol'] @entity35 :: ('10116', 'Species') :: ['Sprague-Dawley rats', 'rats'] @entity67 :: ('MESH:D018754', 'Disease') :: ['diastolic dysfunction'] @entity1121 :: ('MESH:D002312', 'Disease') :: ['HCM', 'Hypertrophic cardiomyopathy'] @entity413 :: ('MESH:D005947', 'Chemical') :: ['glucose'] @entity855 :: ('MESH:D000255', 'Chemical') :: ['ATP']
@entity35 :: (10116,Species) :: ['rat']
