title: Decreased inward rectifier potassium current IK1in dystrophin-deficient ventricular cardiomyocytes.
abstract: Kir2.x channels in ventricular cardiomyocytes (most prominently Kir2.1) account for the inward rectifier potassium current IK1, which controls the resting membrane potential and the final phase of action potential repolarization. Recently it was hypothesized that the dystrophin-associated protein complex (DAPC) is important in the regulation of Kir2.x channels. To test this hypothesis, we investigated potential IK1abnormalities in dystrophin-deficient ventricular cardiomyocytes derived from the hearts of Duchenne muscular dystrophy mouse models. We found that IK1was substantially diminished in dystrophin-deficient cardiomyocytes when compared to wild type myocytes. This finding represents the first functional evidence for a significant role of the DAPC in the regulation of Kir2.x channels.
authors: a Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology , Medical University of Vienna , Vienna , Austria.
authors: Lena
authors: L
authors: Rubi
authors: a Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology , Medical University of Vienna , Vienna , Austria.
authors: Xaver
authors: X
authors: Koenig
authors: a Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology , Medical University of Vienna , Vienna , Austria.
authors: Helmut
authors: H
authors: Kubista
authors: a Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology , Medical University of Vienna , Vienna , Austria.
authors: Hannes
authors: H
authors: Todt
authors: a Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology , Medical University of Vienna , Vienna , Austria.
authors: Karlheinz
authors: K
authors: Hilber
mesh_terms: D000818:Animals; D016189:Dystrophin / Q000172:deficiency* / Q000378:metabolism; D005260:Female; D006352:Heart Ventricles / Q000166:cytology*; D015640:Ion Channel Gating; D008297:Male; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D032383:Myocytes, Cardiac / Q000378:metabolism*; D024661:Potassium Channels, Inwardly Rectifying / Q000378:metabolism*
chemical_list: D016189:Dystrophin; C489461:Kir2.1 channel; D024661:Potassium Channels, Inwardly Rectifying
keywords: Duchenne muscular dystrophy; IK1 inward rectifier potassium current; dystrophin-associated protein complex; dystrophin-deficient mouse models; ventricular cardiomyocytes