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The Role of POPDC Proteins in Cardiac Pacemaking and Conduction

National Heart and Lung Institute, Imperial College London, London W12 0NN, UK
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Academic Editor: Karen Ocorr
J. Cardiovasc. Dev. Dis. 2021, 8(12), 160; https://doi.org/10.3390/jcdd8120160
Received: 27 October 2021 / Revised: 17 November 2021 / Accepted: 20 November 2021 / Published: 23 November 2021
The Popeye domain-containing (POPDC) gene family, consisting of Popdc1 (also known as Bves), Popdc2, and Popdc3, encodes transmembrane proteins abundantly expressed in striated muscle. POPDC proteins have recently been identified as cAMP effector proteins and have been proposed to be part of the protein network involved in cAMP signaling. However, their exact biochemical activity is presently poorly understood. Loss-of-function mutations in animal models causes abnormalities in skeletal muscle regeneration, conduction, and heart rate adaptation after stress. Likewise, patients carrying missense or nonsense mutations in POPDC genes have been associated with cardiac arrhythmias and limb-girdle muscular dystrophy. In this review, we introduce the POPDC protein family, and describe their structure function, and role in cAMP signaling. Furthermore, the pathological phenotypes observed in zebrafish and mouse models and the clinical and molecular pathologies in patients carrying POPDC mutations are described. View Full-Text
Keywords: cyclic nucleotide signaling; cyclic nucleotide binding; sinus bradycardia; atrioventricular block; striated muscle; limb-girdle muscular dystrophy cyclic nucleotide signaling; cyclic nucleotide binding; sinus bradycardia; atrioventricular block; striated muscle; limb-girdle muscular dystrophy
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MDPI and ACS Style

Gruscheski, L.; Brand, T. The Role of POPDC Proteins in Cardiac Pacemaking and Conduction. J. Cardiovasc. Dev. Dis. 2021, 8, 160. https://doi.org/10.3390/jcdd8120160

AMA Style

Gruscheski L, Brand T. The Role of POPDC Proteins in Cardiac Pacemaking and Conduction. Journal of Cardiovascular Development and Disease. 2021; 8(12):160. https://doi.org/10.3390/jcdd8120160

Chicago/Turabian Style

Gruscheski, Lena, and Thomas Brand. 2021. "The Role of POPDC Proteins in Cardiac Pacemaking and Conduction" Journal of Cardiovascular Development and Disease 8, no. 12: 160. https://doi.org/10.3390/jcdd8120160

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