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oai:repisalud.isciii.es:20.500.12105/184112024-11-28T14:50:24Zcom_20.500.12105_15322com_20.500.12105_2051col_20.500.12105_16927

00925njm 22002777a 4500 dc Díaz del Moral, Sandra author Barrena, Silvia author Hernández-Torres, Francisco author Aránega, Amelia author Villaescusa, José Manuel author Gómez Doblas, Juan José author Franco, Diego author Jiménez-Navarro, Manuel author Muñoz-Chápuli, Ramón author Carmona, Rita author 2021-07-22 Expression of Wilms' tumor suppressor transcription factor (WT1) in the embryonic epicardium is essential for cardiac development, but its myocardial expression is little known. We have found that WT1 is expressed at low levels in 20-25% of the embryonic cardiomyocytes. Conditional ablation of WT1 using a cardiac troponin T driver (Tnnt2 Cre ) caused abnormal sinus venosus and atrium development, lack of pectinate muscles, thin ventricular myocardium and, in some cases, interventricular septum and cardiac wall defects, ventricular diverticula and aneurisms. Coronary development was normal and there was not embryonic lethality, although survival of adult mutant mice was reduced probably due to perinatal mortality. Adult mutant mice showed electrocardiographic anomalies, including increased RR and QRS intervals, and decreased PR intervals. RNASeq analysis identified differential expression of 137 genes in the E13.5 mutant heart as compared to controls. GO functional enrichment analysis suggested that both calcium ion regulation and modulation of potassium channels are deeply altered in the mutant myocardium. In summary, together with its essential function in the embryonic epicardium, myocardial WT1 expression is also required for normal cardiac development. 10.3389/fcell.2021.683861 2296-634X Frontiers in Cell and Developmental Biology http://hdl.handle.net/10668/4505 34368133 http://hdl.handle.net/20.500.12105/18411 Wilms’ tumor suppressor gene Cardiomyocytes Cardiac development Calcium homeostasis Potassium channels Genes del tumor de Wilms Genes supresores de tumores Miocitos cardíacos Crecimiento y desarrollo Homeostasis Calcio Canales de potasio Deletion of the Wilms' Tumor Suppressor Gene in the Cardiac Troponin-T Lineage Reveals Novel Functions of WT1 in Heart Development