Arginyltransferase regulates alpha cardiac actin function, myofibril formation and contractility during heart development

摘要: Post-translational arginylation mediated by arginyltransferase (Ate1) is essential for cardiovascular development and angiogenesis in mammals directly affects myocardium structure the developing heart. We recently showed that exerts a number of intracellular effects modifying proteins involved functioning actin cytoskeleton cell motility. Here, we investigated role function cardiac myocytes their actin-containing structures during embryogenesis. Biochemical mass spectrometry analyses alpha undergoes at four sites development. Ultrastructural analysis myofibrils wild-type Ate1 knockout mouse hearts absence results defects myofibril delay affect continuity throughout heart, predicting contractility. Comparison derived from embryos revealed abnormal beating patterns. Our demonstrate cell-autonomous myocyte mice lead to severe congenital abnormalities similar those observed human disease, outline new regulation myocytes.