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Trafficking and functional expression of cardiac Na+ channels
- Source :
- Journal of Molecular and Cellular Cardiology. 36:185-193
- Publication Year :
- 2004
- Publisher :
- Elsevier BV, 2004.
-
Abstract
- Voltage-gated Na(+)channels are essential for the amplitude and upstroke velocity of the cardiac action potential, which are important determinants for impulse propagation and impulse conduction velocity of throughout the working myocardium. Mutations in the major cardiac Na(+)channel gene SCN5A have been implicated in rare, familial forms of cardiac arrhythmias, namely LQT3, Brugada syndrome, progressive cardiac conduction disorder and sudden infant death syndrome. It is increasingly recognized that such mutations--apart from changing channel gating characteristics--may also be related to changes in channel protein trafficking and expression. Regulation of ion channel protein expression depends on a fine-tuned balance among various processes, such as gene transcription, RNA processing, protein synthesis, assembly and post-translational modification, the transport to the cell surface, the anchoring to the cytoskeleton, and regulation of endocytosis and controlled degradation of the protein. During the last decade, interest in factors that control the expression level of ion channels and mechanisms that are involved in targeting of channel proteins to specific sub-cellular and membrane domains is increasing. This review focuses on the current knowledge of mechanisms of cardiac Na(+) channel protein trafficking and expression in cardiomyocytes and its relation to Na(+)-channelopathies.
- Subjects :
- medicine.medical_specialty
Heart Diseases
Action Potentials
Ion Channel Protein
NAV1.5 Voltage-Gated Sodium Channel
Biology
Sodium Channels
Heart Conduction System
Internal medicine
medicine
Animals
Humans
RNA, Messenger
Molecular Biology
Ion channel
Brugada syndrome
Membrane Proteins
Heart
Cardiac action potential
Sudden infant death syndrome
medicine.disease
Cell biology
Transport protein
Protein Transport
Endocrinology
Cardiac Conduction Disorder
Mutation
Cardiology and Cardiovascular Medicine
Subjects
Details
- ISSN :
- 00222828
- Volume :
- 36
- Database :
- OpenAIRE
- Journal :
- Journal of Molecular and Cellular Cardiology
- Accession number :
- edsair.doi.dedup.....86bb844570e84020ab1945fadc864e87