title: 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.
authors: Department of Pharmacology, University of Michigan Medical School, Ann Arbor, Michigan 48109; email: lisom@umich.edu.
authors: Heather A
authors: HA
authors: O'Malley
authors: Lori L
authors: LL
authors: Isom
mesh_terms: D001145:Arrhythmias, Cardiac / Q000235:genetics / Q000503:physiopathology; D001321:Autistic Disorder / Q000235:genetics / Q000503:physiopathology; D002448:Cell Adhesion / Q000235:genetics / Q000502:physiology; D053447:Channelopathies / Q000235:genetics* / Q000503:physiopathology*; D004827:Epilepsy / Q000235:genetics / Q000503:physiopathology; D020869:Gene Expression Profiling; D006801:Humans; D009154:Mutation / Q000235:genetics*; D019636:Neurodegenerative Diseases / Q000235:genetics / Q000503:physiopathology; D011499:Protein Processing, Post-Translational / Q000235:genetics / Q000502:physiology; D062546:Voltage-Gated Sodium Channel beta Subunits / Q000235:genetics* / Q000502:physiology*
chemical_list: D062546:Voltage-Gated Sodium Channel beta Subunits
keywords: arrhythmia; cell adhesion; development; epilepsy; neurodegenerative disease; pain