Published online before print July 3, 2003, doi: 10.1161/01.RES.0000084851.60947.B5
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Submitted on March 19, 2003
Revised on June 24, 2003
Accepted on June 24, 2003
Canine Ventricular KCNE2 Expression Resides Predominantly in Purkinje Fibers
Marc Pourrier ;
From the Departments of Medicine (M.P., S.Z., J.E., W.H., S.N.) and Pharmacology (M.P.), University of Montreal; Department of Pharmacology (S.Z., S.N.), McGill University, Montreal, Quebec, Canada.
* To whom correspondence should be addressed. E-mail: nattel{at}IMumontreal.ca.
Mutations in minK-related peptide 1 (MiRP1), the product of the KCNE2 gene, have been associated with malignant ventricular arrhythmia syndromes related to impaired repolarization. MiRP1 interacts with a variety of ion-channel 
-subunits, dysfunction of which could account for arrhythmia syndromes; however, the observation of very low-level expression of MiRP1 in ventricular tissue has led to doubts about its relevance. The specialized His-Purkinje system plays a key role in cardiac electrophysiology and is an important contributor to ventricular arrhythmias related to abnormal repolarization. We examined the relative abundance of MiRP1 in canine Purkinje versus ventricular tissue and found much greater expression at both mRNA and protein levels in Purkinje tissue. Thus, the cardiac Purkinje system is a strong candidate to play a role in arrhythmic syndromes due to MiRP1 abnormalities.
Key words: long-QT syndromes • ion channel • proarrhythmia
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