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Mechanisms and Alterations of Cardiac Ion Channels Leading to Disease: Role of Ankyrin-B in Cardiac Function

1
Dorothy M. Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
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Departments of Physiology and Cell Biology and Internal Medicine, Division of Cardiovascular Medicine, The Ohio State University College of Medicine and Wexner Medical Center, Columbus, OH 43210, USA
*
Author to whom correspondence should be addressed.
Denotes equal contribution.
Biomolecules 2020, 10(2), 211; https://doi.org/10.3390/biom10020211
Received: 2 January 2020 / Revised: 24 January 2020 / Accepted: 28 January 2020 / Published: 31 January 2020
Ankyrin-B (encoded by ANK2), originally identified as a key cytoskeletal-associated protein in the brain, is highly expressed in the heart and plays critical roles in cardiac physiology and cell biology. In the heart, ankyrin-B plays key roles in the targeting and localization of key ion channels and transporters, structural proteins, and signaling molecules. The role of ankyrin-B in normal cardiac function is illustrated in animal models lacking ankyrin-B expression, which display significant electrical and structural phenotypes and life-threatening arrhythmias. Further, ankyrin-B dysfunction has been associated with cardiac phenotypes in humans (now referred to as “ankyrin-B syndrome”) including sinus node dysfunction, heart rate variability, atrial fibrillation, conduction block, arrhythmogenic cardiomyopathy, structural remodeling, and sudden cardiac death. Here, we review the diverse roles of ankyrin-B in the vertebrate heart with a significant focus on ankyrin-B-linked cell- and molecular-pathways and disease. View Full-Text
Keywords: ankyrin-B; ANK2; ion channels; cardiovascular disease ankyrin-B; ANK2; ion channels; cardiovascular disease
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MDPI and ACS Style

Sucharski, H.C.; Dudley, E.K.; Keith, C.B.R.; El Refaey, M.; Koenig, S.N.; Mohler, P.J. Mechanisms and Alterations of Cardiac Ion Channels Leading to Disease: Role of Ankyrin-B in Cardiac Function. Biomolecules 2020, 10, 211.

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