Research Report 2010 - MDC

Figure 2. Cardiac-restricted ablation of PKD1. Upon the onset of pressureoverload PKD1 mutants showed less hypertrophy and a significantreduction in cardiac fibrosis. TAC: Thoracic aortic banding.mutation in the “muscle LIM protein” (MLP) in patientswith HCM. Surprisingly, immunohistochemical analysisrevealed that MLP is mainly localized in the cytosoliccomponent, rather than in the sarcomere, indicatingthat HCM is not exclusively a sarcomeric disease.Furthermore, their data suggested that impairedmechano-sensory stress signaling might be involved inthe pathogenesis of HCM.Nuclear Receptors in Cardiac MetabolismFlorian BlaschkeThe cardiac phenotype of left ventricular hypertrophycaused by genes that are involved in metabolism andenergy production underscores the impact of energeticpathways on cardiac phenotype and function. A betterunderstanding of the role of cardiac energy metabolismand hypertrophic gene expression may consequentlylead to more effective therapeutic strategies for40 Cardiovascular and Metabolic Disease Research