K(ATP) channel gain-of-function leads to increased myocardial L-type Ca(2+) current and contractility in Cantu syndrome

Cantu syndrome (CS) is caused by gain-of-function (GOF) mutations in genes encoding pore-forming (Kir6.1, KCNJ8) and accessory (SUR2, ABCC9) K(ATP) channel subunits. We show that patients with CS, as well as mice with constitutive (cGOF) or tamoxifen-induced (icGOF) cardiac-specific Kir6.1 GOF subun...

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Detalhes bibliográficos
Publicado no:Proc Natl Acad Sci U S A
Principais autores: Levin, Mark D., Singh, Gautam K., Zhang, Hai Xia, Uchida, Keita, Kozel, Beth A., Stein, Phyllis K., Kovacs, Atilla, Westenbroek, Ruth E., Catterall, William A., Grange, Dorothy Katherine, Nichols, Colin G.
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2016
Assuntos:
Biological Sciences
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4914204/
https://ncbi.nlm.nih.gov/pubmed/27247394
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1606465113
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