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Cell Commun Adhes. 2009;16(1-3):15-27. doi: 10.1080/15419060903009329.

Arrhythmogenic right ventricular cardiomyopathy plakophilin-2 mutations disrupt desmosome assembly and stability.

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1
Department of Oral Biology, University of Nebraska Medical Center College of Dentistry, Lincoln, Nebraska 68583, USA.

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by life-threatening ventricular arrhythmias and fibrofatty replacement of the cardiac tissue. Desmosomes are prominent cell-cell junctions found in a variety of tissues that resist mechanical stress, including the heart, and recruit the intermediate filament cytoskeleton to sites of cell-cell contact. Mutations in several desmosomal components including plakophilin-2 have been identified in ARVC patients; however, the molecular interactions disrupted by plakophilin-2 mutations are currently unknown. To understand the pathological basis of ARVC, the authors analyzed desmosome assembly and stability in epithelial cell lines expressing mutants of plakophilin-2 found in ARVC patients. Mutant plakophilin-2 proteins were unable to disrupt established desmosomes when expressed in an E-cadherin-expressing epithelial cell model; however, they were unable to initiate de novo assembly of desmosomes in an N-cadherin-expressing epithelial cell model. These studies expand our understanding of desmosome assembly and dynamics.

PMID:
19533476
DOI:
10.1080/15419060903009329
[Indexed for MEDLINE]
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