
The hERG gene encodes the pore-forming α-subunit of the rapidly activating delayed rectifier potassium channel (I Kr), which is important for cardiac repolarization. Reduction of I hERG due to genetic mutations or drug interferences causes long QT syndrome, leading to life-threatening cardiac arrhythmias (torsades de pointes) or sudden death. Probucol is a cholesterol-lowering drug that could reduce hERG current by decreasing plasma membrane hERG protein expression and eventually cause long QT syndrome. Here, we investigated the mechanisms of probucol effects on I hERG and hERG-channel expression. Our data demonstrated that probucol reduces SGK1 expression, known as SGK isoform, in a concentration-dependent manner, resulting in downregulation of phosphorylated E3 ubiquitin ligase Nedd4-2 expression, but not the total level of Nedd4-2. As a result, the hERG protein reduces, due to the enhanced ubiquitination level. On the contrary, carbachol could enhance the phosphorylation level of Nedd4-2 as an alternative to SGK1, and thus rescue the ubiquitin-mediated degradation of hERG channels caused by probucol. These discoveries provide a novel mechanism of probucol-induced hERG-channel deficiency, and imply that carbachol or its analog may serve as potential therapeutic compounds for the handling of probucol cardiotoxicity.
ERG1 Potassium Channel, Drug Design, Development and Therapy, Dose-Response Relationship, Drug, Endosomal Sorting Complexes Required for Transport, Anticholesteremic Agents, Nedd4 Ubiquitin Protein Ligases, Ubiquitin-Protein Ligases, Protein Serine-Threonine Kinases, Ether-A-Go-Go Potassium Channels, Immediate-Early Proteins, Long QT Syndrome, HEK293 Cells, Probucol, Humans, Carbachol, Phosphorylation, Serum-Glucocorticoid Regulated Kinases, Original Research
ERG1 Potassium Channel, Drug Design, Development and Therapy, Dose-Response Relationship, Drug, Endosomal Sorting Complexes Required for Transport, Anticholesteremic Agents, Nedd4 Ubiquitin Protein Ligases, Ubiquitin-Protein Ligases, Protein Serine-Threonine Kinases, Ether-A-Go-Go Potassium Channels, Immediate-Early Proteins, Long QT Syndrome, HEK293 Cells, Probucol, Humans, Carbachol, Phosphorylation, Serum-Glucocorticoid Regulated Kinases, Original Research
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