DOI: 10.5176/2382-607X_ARP14.34

Authors: Jim J.-C. Lin, Qinchuan Wang & Jenny L.-C. Lin, F.-C. Chan, K.-H. Wu, P.-H. Jiang & C.-I Lin

Abstract: The Xin repeat-containing proteins, Xin and Xin, are intercalated disc (ICD)-associated scaffolding proteins, playing important roles in ICD maturation and integrity as well as in cardiac growth and rhythms. Mice deficient in Xin (mXin) develop progressive cardiac hypertrophy and cardiomyopathy with conduction defects, whereas mice totally lacking mXin exhibit severe growth retardation, abnormal heart shape, ventricular septal defects and failure of forming ICDs, diastolic dysfunction, atrioventricular (AV) conduction block, and postnatal lethality. Here, we discuss what we have known of Xins’ functions and their interacting partners, and how the loss of mXin proteins would lead to these cardiac defects. We hope a better understanding of the pathogenesis will enable the assessment of novel therapeutic interventions for these diseases.

Keywords: intercalated disc maturation, XIRP1, XIRP2, atrial fibrillation, atrioventricular conduction block

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