Biophysical Investigation of Sodium Channel Interaction with β-Subunit Variants Associated with Arrhythmias.

ABSTRACT: Background: Voltage-gated sodium (Na_V) channels help regulate electrical activity of the plasma membrane. Mutations in associated subunits can result in pathological outcomes. Here we examined the interaction of Na_V channels with cardiac arrhythmia-linked mutations in SCN2B and SCN4B, two genes that encode auxiliary β-subunits. Materials and Methods: To investigate changes in SCN2B ^R137H and SCN4B ^I80T function, we combined three-dimensional X-ray crystallography with electrophysiological measurements on Na_V1.5, the dominant subtype in the heart. Results: SCN4B ^I80T alters channel activity, whereas SCN2B ^R137H does not have an appa

SUBMITTER: Llongueras JP 

PROVIDER: S-EPMC8370339 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature