Arrhythmogenic Cardiomyopathy: Pathophysiology Beyond Cardiac Myocytes.

In the current issue of the Circulation Research, Karmouch et
al1 present a provocative study attributing a pivotal role in the
pathogenesis of arrhythmogenic cardiomyopathy (ACM) to the
loss of DSP (desmoplakin) gene in a subpopulation of the cells
of the conduction system. This hypothesis is in sharp departure
from the current view on the disease, and it opens the question
on whether the data reported in mice with a selective expression
of the genetic defects in the specialized cells of the conduction
system replicate the clinical phenotype found in patients.