Proteomic analyses of transgenic LQT1 and LQT2 rabbit hearts elucidate an increase in expression and activity of energy producing enzymes

► 2-D DIGE was used to analyze the proteomes in long QT syndrome I and II rabbit hearts ► Differentially expressed proteins were identified in long QTS rabbit hearts ► There was increased expression of enzymes involved in ATP generation ► Enzymatic assays and western blot analyses validated 2-D DIGE LC-MS/MS results