Na+ channel protein, 4-beta subunit (SCN4B)
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Function
- modulates channel gating kinetics of the voltage-gated Na+ channel
- causes negative shifts in the voltage dependence of activation of certain alpha Na+ channels, but does not affect the voltage dependence of inactivation (putative)
- contains a number of interchain disulfide bonds with SCN2A
- the voltage-sensitive Na+ channel consists of an ion conducting pore forming alpha-subunit regulated by one or more beta-1, beta-2, beta-3 &/or beta-4 subunits
- beta-1 & beta-3 are non-covalently associated with alpha, while beta-2 & beta-4 are covalently linked by disulfide bonds.
- associates with SCN2A
Structure
- contains 1 Ig-like C2-type domain (immunoglobulin-like)
Compartment
- membrane; single-pass type 1 membrane protein (probable)
Alternative splicing
named isoforms=2
Expression
- expressed at a high level in dorsal root ganglia, at a lower level in brain, spinal cord, skeletal muscle & heart
Pathology
- defects in SCN4B are the cause of long QT syndrome type 10 (LQT10)