[Anchoring proteins and cardiac sudden death: how and why?].
Mutations in proteins responsible for ion transport in cardiac tissue can induce a destabilization of electrical function and provoke cardiac sudden death. Identification of a genetic anomaly in a French family that developed the syndrome of cardiac sudden death has revealed a crucial new element in normal cardiac electrical function : Ion channels need to be anchored to specific domains at the plasma membrane by an anchoring protein called ankyrin-B.
Pourrier
Marc
M
Département de pharmacologie et Centre de recherche, Université de Montréal, Québec, Canada.
Nattel
Stanley
S
D000200:Action Potentials; D000818:Animals; D017089:Ankyrin Repeat; D017487:Ankyrins / Q000737:chemistry / Q000172:deficiency* / Q000235:genetics / Q000502:physiology; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology; D002118:Calcium / Q000502:physiology; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D006329:Heart Conduction System / Q000503:physiopathology; D006801:Humans; D007473:Ion Channels / Q000235:genetics / Q000502:physiology; D017136:Ion Transport / Q000235:genetics; D008133:Long QT Syndrome / Q000235:genetics / Q000503:physiopathology; D051379:Mice; D018345:Mice, Knockout; D008954:Models, Biological; D012964:Sodium / Q000502:physiology
C498994:ANK2 protein, human; C498995:Ank2 protein, mouse; D017487:Ankyrins; D007473:Ion Channels; D012964:Sodium; D002118:Calcium