Molecular mechanisms in the pathogenesis of arrhythmogenic cardiomyopathy.
The article is based on work presented in the Distinguished Achievement Award lecture at the Society for Cardiovascular Pathology meeting in Seattle, WA, in March 2016. It reviews our current understanding of mechanisms responsible for a highly arrhythmogenic, nonischemic cardiomyopathy. It highlights the armamentarium of powerful methods available to the experimental pathologist in efforts to define how complex cardiovascular diseases work. It concludes with acknowledgment of the need for a far more detailed approach as to how we categorize human disease, a task for which pathologists are especially well positioned.
Department of Pathology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA. Electronic address: jsaffitz@bidmc.harvard.edu.
Jeffrey E
JE
Saffitz
D000818:Animals; D030801:Animals, Genetically Modified; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000235:genetics / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D004195:Disease Models, Animal; D005838:Genotype; D000071679:Glycogen Synthase Kinase 3 beta / Q000037:antagonists & inhibitors / Q000378:metabolism; D006801:Humans; D007211:Indoles / Q000494:pharmacology; D007473:Ion Channels / Q000235:genetics / Q000378:metabolism*; D008301:Maleimides / Q000494:pharmacology; D009061:Mouth Mucosa / Q000378:metabolism / Q000473:pathology; D032383:Myocytes, Cardiac / Q000378:metabolism* / Q000473:pathology; D010641:Phenotype; D047428:Protein Kinase Inhibitors / Q000494:pharmacology; D021381:Protein Transport; D060449:Wnt Signaling Pathway; D015027:Zebrafish / Q000235:genetics / Q000378:metabolism
D007211:Indoles; D007473:Ion Channels; D008301:Maleimides; D047428:Protein Kinase Inhibitors; C417521:SB 216763; D000071679:Glycogen Synthase Kinase 3 beta
Arrhythmogenic cardiomyopathy; Buccal mucosa; Desmosomes; Gap junctions; Ion channels; Protein trafficking; Zebrafish models