Title : Syntrophin mutation associated with long QT syndrome through activation of the nNOS-SCN5A macromolecular complex.

Pub. Date : 2008 Jul 8

PMID : 18591664






4 Functional Relationships(s)
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Compound Name
Protein Name
Organism
1 A390V-SNTA1 expressed with SCN5A, nNOS, and PMCA4b in heterologous cells increased peak and late sodium current compared with WT-SNTA1, and the increase was partially inhibited by NOS blockers. Sodium syntrophin alpha 1 Homo sapiens
2 Expression of A390V-SNTA1 in cardiac myocytes also increased late sodium current. Sodium syntrophin alpha 1 Homo sapiens
3 These results establish an SNTA1-based nNOS complex attached to SCN5A as a key regulator of sodium current and suggest that SNTA1 be considered a rare LQTS-susceptibility gene. Sodium syntrophin alpha 1 Homo sapiens
4 These results establish an SNTA1-based nNOS complex attached to SCN5A as a key regulator of sodium current and suggest that SNTA1 be considered a rare LQTS-susceptibility gene. Sodium syntrophin alpha 1 Homo sapiens