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Article

Inhibition of Late Sodium Current by Mexiletine

12 Apr 2013

Abstract excerpt

BACKGROUND: Timothy syndrome (TS) is a rare long-QT syndrome caused by CACNA1C mutations G406R in exon 8A (TS1) and G402S/G406R in exon 8 (TS2). Management of TS is a challenge and prognosis is poor. This study aimed to explore the inheritance pattern and mechanism of an INa blocker, mexiletine, to improve clinical manifestations in TS. METHODS AND RESULTS: A 2-year-old Chinese girl with a typical TS1 phenotype...

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Share to Cardiac electrophysiology and arrhythmias
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PMID: 23580742
DOI: 10.1161/circep.113.000092

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Related research

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Inhibition of Late Sodium Current by MexiletinePMID 23580742Read ↗

Increased CaV1.2 late current by a CACNA1C p.R412M variant causes an atypical Timothy syndrome without syndactyly.Semantic proximity Read ↗

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