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The junctophilin family of proteins: from bench to bedside.

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Date
2014-06
Authors
Landstrom, AP
Beavers, DL
Wehrens, XHT
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Abstract
Excitable tissues rely on junctional membrane complexes to couple cell surface signals to intracellular channels. The junctophilins have emerged as a family of proteins critical in coordinating the maturation and maintenance of this cellular ultrastructure. Within skeletal and cardiac muscle, junctophilin 1 and junctophilin 2, respectively, couple sarcolemmal and intracellular calcium channels. In neuronal tissue, junctophilin 3 and junctophilin 4 may have an emerging role in coupling membrane neurotransmitter receptors and intracellular calcium channels. These important physiological roles are highlighted by the pathophysiology which results when these proteins are perturbed, and a growing body of literature has associated junctophilins with the pathogenesis of human disease.
Type
Journal article
Subject
Muscle, Skeletal
Myocardium
Neurons
Humans
Muscular Diseases
Cardiomyopathies
Muscle Proteins
Membrane Proteins
Phylogeny
Calcium Signaling
Mutation
Muscle, Striated
Permalink
https://hdl.handle.net/10161/20313
Published Version (Please cite this version)
10.1016/j.molmed.2014.02.004
Publication Info
Landstrom, AP; Beavers, DL; & Wehrens, XHT (2014). The junctophilin family of proteins: from bench to bedside. Trends in molecular medicine, 20(6). pp. 353-362. 10.1016/j.molmed.2014.02.004. Retrieved from https://hdl.handle.net/10161/20313.
This is constructed from limited available data and may be imprecise. To cite this article, please review & use the official citation provided by the journal.
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Scholars@Duke

Landstrom

Andrew Paul Landstrom

Associate Professor of Pediatrics
Dr. Landstrom is a physician scientist who specializes in the care of children and young adults with arrhythmias, heritable cardiovascular diseases, and sudden unexplained death syndromes. As a clinician, he is trained in pediatric cardiology with a focus on arrhythmias and genetic diseases of the heart.  He specializes in caring for patients with heritable arrhythmia (channelopathies) such as long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia,
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