Mechanisms of human arrhythmia syndromes: abnormal cardiac macromolecular interactions.
Many cardiac ion channels exist within macromolecular signaling complexes, comprised of pore-forming subunits that associate with auxiliary subunits, regulatory enzymes, and targeting proteins. This complex protein assembly ensures proper modulation of channel activity and ion homeostasis. The association of genetic defects in regulatory and targeting proteins to inherited arrhythmia syndromes has led to a better understanding of the critical role these proteins play in ion channel modulation.
Mohler
Peter J
PJ
Department of Internal Medicine, Division of Cardiology, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
Wehrens
Xander H T
XH
D000818:Animals; D017487:Ankyrins / Q000235:genetics / Q000378:metabolism; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000235:genetics / Q000378:metabolism; D022461:Caveolins / Q000235:genetics / Q000378:metabolism; D006801:Humans; D015640:Ion Channel Gating / Q000235:genetics; D046911:Macromolecular Substances / Q000037:antagonists & inhibitors / Q000378:metabolism*; D009154:Mutation / Q000502:physiology; D032383:Myocytes, Cardiac / Q000378:metabolism / Q000648:ultrastructure; D021122:Protein Subunits / Q000235:genetics / Q000378:metabolism; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics / Q000378:metabolism; D015398:Signal Transduction / Q000235:genetics
D017487:Ankyrins; D022461:Caveolins; D046911:Macromolecular Substances; D021122:Protein Subunits; D019837:Ryanodine Receptor Calcium Release Channel