Pharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum

KCNH2 encodes Kv11.1 and underlies the rapidly activating delayed rectifier K(+) current (I(Kr)) in the heart. Loss-of-function KCNH2 mutations cause the type 2 long QT syndrome (LQT2), and most LQT2-linked missense mutations inhibit the trafficking of Kv11.1 channels. Drugs that bind to Kv11.1 and...

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Hauptverfasser: Smith, Jennifer L., Reloj, Allison R., Nataraj, Parvathi S., Bartos, Daniel C., Schroder, Elizabeth A., Moss, Arthur J., Ohno, Seiko, Horie, Minoru, Anderson, Corey L., January, Craig T., Delisle, Brian P.
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Physiological Society 2013
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Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4042535/
https://ncbi.nlm.nih.gov/pubmed/23864605
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajpcell.00406.2012
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