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The Timothy syndrome mutation of cardiac CaV1.2 (L-type) channels: multiple altered gating mechanisms and pharmacological restoration of inactivation

Timothy syndrome (TS) is a multiorgan dysfunction caused by a Gly to Arg substitution at position 406 (G406R) of the human CaV1.2 (L-type) channel. The TS phenotype includes severe arrhythmias that are thought to be triggered by impaired open-state voltage-dependent inactivation (OSvdI). The effect...

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Detalhes bibliográficos
Main Authors: Yarotskyy, Viktor, Gao, Guofeng, Peterson, Blaise Z, Elmslie, Keith S
Formato: Artigo
Idioma:Inglês
Publicado em: Blackwell Science Inc 2009
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2670080/
https://ncbi.nlm.nih.gov/pubmed/19074970
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1113/jphysiol.2008.161737
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