Commentary 10.1172/JCI135759

The surprising complexity of KATP channel biology and of genetic diseases

Guiling Zhao,1 Aaron Kaplan,1,2 Maura Greiser,1 and W. Jonathan Lederer1

1Center for Biomedical Engineering and Technology and Department of Physiology,

2Department of Medicine and Division of Cardiology, University of Maryland Baltimore, School of Medicine, Baltimore, Maryland, USA.

Address correspondence to: W. Jonathan Lederer, University of Maryland Baltimore, School of Medicine, 111 S. Penn Street, Baltimore, Maryland 21201, USA. Phone: 410.706.4895; Email: jlederer@som.umaryland.edu.

Find articles by Zhao, G. in: JCI | PubMed | Google Scholar

1Center for Biomedical Engineering and Technology and Department of Physiology,

2Department of Medicine and Division of Cardiology, University of Maryland Baltimore, School of Medicine, Baltimore, Maryland, USA.

Address correspondence to: W. Jonathan Lederer, University of Maryland Baltimore, School of Medicine, 111 S. Penn Street, Baltimore, Maryland 21201, USA. Phone: 410.706.4895; Email: jlederer@som.umaryland.edu.

Find articles by Kaplan, A. in: JCI | PubMed | Google Scholar |

1Center for Biomedical Engineering and Technology and Department of Physiology,

2Department of Medicine and Division of Cardiology, University of Maryland Baltimore, School of Medicine, Baltimore, Maryland, USA.

Address correspondence to: W. Jonathan Lederer, University of Maryland Baltimore, School of Medicine, 111 S. Penn Street, Baltimore, Maryland 21201, USA. Phone: 410.706.4895; Email: jlederer@som.umaryland.edu.

Find articles by Greiser, M. in: JCI | PubMed | Google Scholar

1Center for Biomedical Engineering and Technology and Department of Physiology,

2Department of Medicine and Division of Cardiology, University of Maryland Baltimore, School of Medicine, Baltimore, Maryland, USA.

Address correspondence to: W. Jonathan Lederer, University of Maryland Baltimore, School of Medicine, 111 S. Penn Street, Baltimore, Maryland 21201, USA. Phone: 410.706.4895; Email: jlederer@som.umaryland.edu.

Find articles by Lederer, W. in: JCI | PubMed | Google Scholar |

First published February 17, 2020 - More info

Published in Volume 130, Issue 3 on March 2, 2020
J Clin Invest. 2020;130(3):1112–1115. https://doi.org/10.1172/JCI135759.
© 2020 American Society for Clinical Investigation
First published February 17, 2020 - Version history

The ATP-sensitive K+ channel (KATP) is formed by the association of four inwardly rectifying K+ channel (Kir6.x) pore subunits with four sulphonylurea receptor (SUR) regulatory subunits. Kir6.x or SUR mutations result in KATP channelopathies, which reflect the physiological roles of these channels, including but not limited to insulin secretion, cardiac protection, and blood flow regulation. In this issue of the JCI, McClenaghan et al. explored one of the channelopathies, namely Cantu syndrome (CS), which is a result of one kind of KATP channel mutation. Using a knockin mouse model, the authors demonstrated that gain-of-function KATP mutations in vascular smooth muscle resulted in cardiac remodeling. Moreover, they were able to reverse the cardiovascular phenotypes by administering the KATP channel blocker glibenclamide. These results exemplify how genetic mutations can have an impact on developmental trajectories, and provide a therapeutic approach to mitigate cardiac hypertrophy in cases of CS.

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