Acta Pharmacologica Sinica (2010) 31: 1019–1021; doi: 10.1038/aps.2010.116

Acta Pharmacologica Sinica (2010) 31: 1019–1021; doi: 10.1038/aps.2010.116; published online 9 Aug 2010

Perspective

Emerging role of junctophilin-2 as a regulator of calcium handling in the heart

Alejandro GARBINO^{1, 2}, Xander HT WEHRENS^{1, 2, 3, *}

¹Department of Molecular Physiology and Biophysics, ²Translational Biology and Molecular Medicine Program, ³Department of Medicine-Division of Cardiology, Baylor College of Medicine, Houston, Texas, USA

Junctophilin-2 (JPH2) is a membrane-binding protein that plays a key role in the organization of the junctional membrane complex (JMC) in cardiac myocytes. JPH2 is believed to keep the plasma membrane and sarcoplasmic reticulum at a fixed distance within the JMC, which is essential for proper Ca²⁺-induced Ca²⁺ release during the excitation-contraction process. Recent studies have revealed that mutations in the JPH2 gene are associated with hypertrophic cardiomyopathy, highlighting the importance of this protein for normal cardiac physiology. In this paper, we review current knowledge about the structure and function of junctophilin-2 in the heart.

Keywords: Ca²⁺-induced Ca²⁺ release; excitation contraction coupling; heart failure; junctional membrane complex; junctophilin-2; RyR2

Xander HT WEHRENS is a WM Keck Foundation Distinguished Young Scholar in Medical Research, and is supported by NIH/NHLBI grants R01-HL089598 and R01-HL091947, Muscular Dystrophy Association grant #69238, and the Fondation Leducq Award to the ‘Alliance for Calmodulin Kinase Signaling in Heart Disease’.

^*To whom correspondence should be addressed.
E-mail wehrens@bcm.edu
Received 2010-06-04 Accepted 2010-07-08

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