scholarly journals Erratum: Corrigendum: Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy

2005 ◽  
Vol 37 (1) ◽  
pp. 106-106 ◽  
Author(s):  
B Gerull ◽  
A Heuser ◽  
T Wichter ◽  
M Paul ◽  
C T Basson ◽  
...  
2004 ◽  
Vol 36 (11) ◽  
pp. 1162-1164 ◽  
Author(s):  
Brenda Gerull ◽  
Arnd Heuser ◽  
Thomas Wichter ◽  
Matthias Paul ◽  
Craig T Basson ◽  
...  

2006 ◽  
Vol 70 (7) ◽  
pp. 933-935 ◽  
Author(s):  
Iori Nagaoka ◽  
Keiji Matsui ◽  
Takeshi Ueyama ◽  
Masashi Kanemoto ◽  
Jie Wu ◽  
...  

Author(s):  
Perry Elliott ◽  
Kristina H. Haugaa ◽  
Pio Caso ◽  
Maja Cikes

Restrictive cardiomyopathy is a heart muscle disorder characterized by increased myocardial stiffness that results in an abnormally steep rise in intraventricular pressure with small increases in volume in the presence of normal or decreased diastolic left ventricular volumes and normal ventricular wall thickness. The disease may be caused by mutations in a number of genes or myocardial infiltration. Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiac muscle disease associated with sudden cardiac death, ventricular arrhythmias, and cardiac failure. It is most frequently caused by mutations in desmosomal protein genes that lead to fibrofatty replacement of cardiomyocytes, right ventricular dilatation, and aneurysm formation.


2010 ◽  
Vol 107 (6) ◽  
pp. 700-714 ◽  
Author(s):  
Mario Delmar ◽  
William J. McKenna

Intercellular communication is essential for proper cardiac function. Mechanical and electrical activity need to be synchronized so that the work of individual myocytes transforms into the pumping function of the organ. Mechanical continuity is provided by desmosomes and adherens junctions, while gap junctions provide a pathway for passage of ions and small molecules between cells. These complexes preferentially reside at the site of end-end contact between myocytes, within the intercalated disc. Recognition that some forms of arrhythmogenic cardiomyopathy are caused by mutations in desmosomal protein genes has galvanized interest in the biology of the desmosome and its interactions with other junctional molecules. This review presents the cellular and molecular biology of the desmosome, current knowledge on the relation of desmosomal mutations and disease phenotypes, and an overview of the molecular pathophysiology of arrhythmogenic right ventricular cardiomyopathy. Clinical experience and results from cellular and animal models provide insights into the intercalated disc as a functional unit and into the basic substrates that underlie pathogenesis and arrhythmogenesis of arrhythmogenic right ventricular cardiomyopathy.


Cardiology ◽  
2021 ◽  
Author(s):  
Anneli Svensson ◽  
Pyotr G. Platonov ◽  
Kristina H. Haugaa ◽  
Wojciech Zareba ◽  
Henrik Kjærulf Jensen ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document