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Sodium channel β subunits: emerging targets in channelopathies.

Abstract
Voltage-gated sodium channels (VGSCs) are responsible for the initiation and propagation of action potentials in excitable cells. VGSCs in mammalian brain are heterotrimeric complexes of α and β subunits. Although β subunits were originally termed auxiliary, we now know that they are multifunctional signaling molecules that play roles in both excitable and nonexcitable cell types and with or without the pore-forming α subunit present. β subunits function in VGSC and potassium channel modulation, cell adhesion, and gene regulation, with particularly important roles in brain development. Mutations in the genes encoding β subunits are linked to a number of diseases, including epilepsy, sudden death syndromes like SUDEP and SIDS, and cardiac arrhythmia. Although VGSC β subunit-specific drugs have not yet been developed, this protein family is an emerging therapeutic target.
AuthorsHeather A O'Malley, Lori L Isom
JournalAnnual review of physiology (Annu Rev Physiol) Vol. 77 Pg. 481-504 ( 2015) ISSN: 1545-1585 [Electronic] United States
PMID25668026 (Publication Type: Journal Article, Review)
Chemical References
  • Voltage-Gated Sodium Channel beta Subunits
Topics
  • Arrhythmias, Cardiac (genetics, physiopathology)
  • Autistic Disorder (genetics, physiopathology)
  • Cell Adhesion (genetics, physiology)
  • Channelopathies (genetics, physiopathology)
  • Epilepsy (genetics, physiopathology)
  • Gene Expression Profiling
  • Humans
  • Mutation (genetics)
  • Neurodegenerative Diseases (genetics, physiopathology)
  • Protein Processing, Post-Translational (genetics, physiology)
  • Voltage-Gated Sodium Channel beta Subunits (genetics, physiology)

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