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A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the Ikr potassium channel

Article Abstract:

A major subunit of the cardiac IKr potassium channel is encoded by human ether-a-go-go-related gene (HERG). HERG, when expressed in Xenopus oocytes, shows biophysical characteristics similar to the rapidly activating delayed rectifier potassium current (IKr) in cardiac myocytes. The HERG current has been found to be selective for potassium, and a decline in the current is seen at depolarizations above 0 mV. Extracellular potassium ions can activate this current. Lanthanum and cobalt inhibit the HERG current. The results suggest a mechanistic connection between inherited and acquired forms of long QT syndrome, which causes cardiac arrhythmias.

Author: Sanguinetti, Michael C., Keating, Mark T., Curran, Mark E., Jiang, Changan
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1995
Heart diseases, Potassium channels

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Cav1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and Autism

Article Abstract:

Timothy syndrome, a novel disorder characterized by multiorgan dysfunction including lethal arrhythmias, webbing of fingers and toes, congenital heart disease, immune deficiency, intermittent hypoglycemia, cognitive abnormalities, and autism is reported. The Cav1.2 the cardiac L-type calcium channel is important for excitation and contraction of the heart.

Author: Schwartz, Peter J., Priori, Silvia G., Napolitano, Carlo, Bloise, Raffaella, Keating, Mark T., Splwski, Igor., Decher, Niels, Joseph, Robert M., Tager-Flusberg, Helen, Sharpe, Leah M., Kumar, Pradeep, Condouris, Karen, Sanguinetti,Michael C.
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2004
Science & research, Causes of, Autism

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Subjects list: Research, Arrhythmia
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