Role of Genetic Analysis in the Management of Patients With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Srijita Sen-Chowdhry, MBBS, MD, MRCP,
Petros Syrris, BSc, PhD and
William J. McKenna, MD, DSc, FRCP, FACC*
Cardiology in the Young, The Heart Hospital, University College London, London, United Kingdom
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Figure 1 Schematic of Desmosomal Structure
The desmosomal cadherins, desmoglein (DSG)-2 and desmocollin (DSC)-2 comprise the transmembrane component of the desmosomal complex. Their extracellular domains interface directly with their counterparts on neighboring cells. The intracellular portions of the desmosomal cadherins interact with proteins of the armadillo family: junctional plakoglobin (JUP) and plakophilin (PKP)-2, which in turn bind to the N-terminal domain of desmoplakin (DSP). At its C-terminal, DSP anchors desmin intermediate filaments to the cell surface. Modified from Sen-Chowdhry et al. (8).
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