Dysfunction in the βII spectrin-dependent cytoskeleton underlies human arrhythmia.

ABSTRACT:

Background

The cardiac cytoskeleton plays key roles in maintaining myocyte structural integrity in health and disease. In fact, human mutations in cardiac cytoskeletal elements are tightly linked to cardiac pathologies, including myopathies, aortopathies, and dystrophies. Conversely, the link between cytoskeletal protein dysfunction and cardiac electric activity is not well understood and often overlooked in the cardiac arrhythmia field.

Methods and results

Here, we uncover a new mechanism for the regulation of cardiac membrane excitability. We report that βII spectrin, an actin-associated molecule, is essential for the posttranslational targeting and localization of critical membrane proteins in heart. βII spectrin recruits ankyrin-B to the cardiac dyad, and a novel human m

SUBMITTER: Smith SA 

PROVIDER: S-EPMC4342332 | biostudies-literature | 2015 Feb

REPOSITORIES: biostudies-literature