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Sodium channel ? subunits: emerging targets in channelopathies
#MMPMID25668026
O'Malley HA
; Isom LL
Annu Rev Physiol
2015[]; 77
(?): 481-504
PMID25668026
show ga
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 ? and ? subunits. Although ? 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 ? subunit present. ? 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 ? subunits
are linked to a number of diseases, including epilepsy, sudden death syndromes
like SUDEP and SIDS, and cardiac arrhythmia. Although VGSC ? subunit-specific
drugs have not yet been developed, this protein family is an emerging therapeutic
target.
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