A little too much : cardiac electrophysiological effects of elevated inward rectifying current carried by the Kir2.1 ion channel protein
| Autor: | Marlieke G. Veldhuis, Yuan Ji, Marcel A.G. van der Heyden |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2015 |
| Předmět: |
medicine.medical_specialty
biology Inward-rectifier potassium ion channel Chemistry Cardiac electrophysiology Kir2.1 Skeletal muscle Atrial fibrillation Short QT syndrome General Medicine medicine.disease Electrophysiology Endocrinology medicine.anatomical_structure Internal medicine biology.protein medicine KvLQT1 |
| Zdroj: | Adaptive Medicine, 7, 1. Taipei : Society of Adaptive Science in Taiwan |
| ISSN: | 2076-944X |
| Popis: | Inward rectifier currents carried by K_(IR)2.1 proteins have an important role in cardiac electrophysiology. Animal knock-outs and human loss-of-function mutation carriers experience cardiac pro-arrhythmia, but phenotypes are not confined to the heart since these channels are prominently expressed in many other organs and tissues. We here review the other end of the spectrum, in which gainof- function of the K_(IR)2.1 carried I_(K1) results in action potential shortening in isolated cardiomyocytes, and QT shortening in animals and humans. Gain-of-function mutations in patients often result in short QT syndrome accompanied with atrial fibrillation. Remarkable, skeletal muscle, neurological and developmental abnormalities are less prominent in these patients compared to their loss-of-function counterparts. Finally, the most common pathological arrhythmia, atrial fibrillation, is associated with K_(IR)2.1 upregulation at the mRNA and protein level, and concomitant enhanced I_(K1) density in atrial tissues. |
| Databáze: | OpenAIRE |
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