A protein-degradation mechanism opens a new treatment route for an inherited arrhythmia
An international team has identified a protein-degradation mechanism that contributes to the development of an inherited form of catecholaminergic polymorphic ventricular tachycardia (CPVT), a disease that mainly affects children and young people. The study was led by researchers at the Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), in collaboration with the University of…
An international research team, including scientists from the Spanish cardiovascular research network, has uncovered a protein-degradation mechanism implicated in an inherited form of arrhythmia called catecholaminergic polymorphic ventricular tachycardia (CPVT). CPVT, which mainly affects children and young adults, can cause life-threatening heart rhythm disturbances during physical exertion or emotional stress.
Led by Dr. Enrique Lara-Pezzi of the CNIC, the study, published in Circulation Research, reveals that mutations in the gene encoding calsequestrin, a calcium-regulating protein, trigger cellular degradation pathways that diminish crucial proteins in the calcium-release machinery. Specifically, the enzyme calpain, which becomes more active in affected individuals, targets a protein called triadin, leading to destabilization of the calcium-release system.
The researchers found that inhibiting calpain restored triadin levels, improved calcium handling, and reduced ventricular arrhythmias in mice with the mutation. While these findings suggest that calpain inhibition could be a new therapeutic target for CPVT, the authors caution that more research is needed to determine its applicability to other forms of the disease and potential human use.
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