Format

Send to

Choose Destination
Handb Exp Pharmacol. 2018;246:423-450. doi: 10.1007/164_2017_48.

Voltage-Gated Sodium Channel β Subunits and Their Related Diseases.

Author information

1
Department of Pharmacology, University of Michigan Medical School, 2200 MSRBIII, 1150 W. Medical Center Dr., Ann Arbor, MI, 48109-5632, USA.
2
Department of Pharmacology, University of Michigan Medical School, 2301 MSRB III, 1150 W. Medical Center Dr., Ann Arbor, MI, 48109-5632, USA. lisom@umich.edu.

Abstract

Voltage-gated sodium channels are protein complexes comprised of one pore forming α subunit and two, non-pore forming, β subunits. The voltage-gated sodium channel β subunits were originally identified to function as auxiliary subunits, which modulate the gating, kinetics, and localization of the ion channel pore. Since that time, the five β subunits have been shown to play crucial roles as multifunctional signaling molecules involved in cell adhesion, cell migration, neuronal pathfinding, fasciculation, and neurite outgrowth. Here, we provide an overview of the evidence implicating the β subunits in their conducting and non-conducting roles. Mutations in the β subunit genes (SCN1B-SCN4B) have been linked to a variety of diseases. These include cancer, epilepsy, cardiac arrhythmias, sudden infant death syndrome/sudden unexpected death in epilepsy, neuropathic pain, and multiple neurodegenerative disorders. β subunits thus provide novel therapeutic targets for future drug discovery.

KEYWORDS:

Cell adhesion; Channelopathy; Dravet syndrome; Neuronal pathfinding; Sudden unexpected death in epilepsy; Voltage-gated sodium channel; α subunit; β subunit

PMID:
28965169
DOI:
10.1007/164_2017_48
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Springer
Loading ...
Support Center