Voltage-Dependent Sarcolemmal Ion Channel Abnormalities in the Dystrophin-Deficient Heart

International Journal of Molecular Sciences

Xaver Koenig¹  Janine Ebner¹  Karlheinz Hilber¹ 

[1] Department of Neurophysiology and—Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Schwarzspanierstraße 17, 1090 Vienna, Austria;

关键词: animal models;    calcium channels;    cardiac ion channel abnormalities;    dystrophic cardiomyopathy;    dystrophin deficiency;    dystrophinopathies;    potassium channels;    sodium channels;   

DOI  :  10.3390/ijms19113296

来源: DOAJ

【 摘 要 】

Mutations in the gene encoding for the intracellular protein dystrophin cause severe forms of muscular dystrophy. These so-called dystrophinopathies are characterized by skeletal muscle weakness and degeneration. Dystrophin deficiency also gives rise to considerable complications in the heart, including cardiomyopathy development and arrhythmias. The current understanding of the pathomechanisms in the dystrophic heart is limited, but there is growing evidence that dysfunctional voltage-dependent ion channels in dystrophin-deficient cardiomyocytes play a significant role. Herein, we summarize the current knowledge about abnormalities in voltage-dependent sarcolemmal ion channel properties in the dystrophic heart, and discuss the potentially underlying mechanisms, as well as their pathophysiological relevance.

【 授权许可】

Unknown