scholarly journals The Trafficking of Mutant HERG K+ Channels Linked to Long QT Syndrome are Regulated by a Subdomain in the Endoplasmic Reticulum

2011 ◽  
Vol 100 (3) ◽  
pp. 427a
Author(s):  
Jennifer L. Smith ◽  
Parvathi Nataraj ◽  
Craig T. January ◽  
Brian P. Delisle
Keyword(s):  
Endoplasmic Reticulum ◽  
Long Qt Syndrome ◽  
Long Qt ◽  
K Channels ◽  
Qt Syndrome

Retention in the Endoplasmic Reticulum as a Mechanism of Dominant-negative Current Suppression in Human Long QT Syndrome

2000 ◽  
Vol 32 (12) ◽  
pp. 2327-2337 ◽  
Author(s):  
Eckhard Ficker ◽  
Adrienne T Dennis ◽  
Carlos A Obejero-Paz ◽  
Pasqualina Castaldo ◽  
Maurizio Taglialatela ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Long Qt Syndrome ◽  
Dominant Negative ◽  
Long Qt ◽  
Negative Current ◽  
Qt Syndrome

scholarly journals Trafficking Deficient KV11.1 (HERG) Mutations Linked to Long QT Syndrome Localize to Different Endoplasmic Reticulum Subcompartments

2016 ◽  
Vol 110 (3) ◽  
pp. 105a
Author(s):  
Jennifer L. Smith ◽  
Corey L. Anderson ◽  
Criag T. January ◽  
Brian Delisle
Keyword(s):  
Endoplasmic Reticulum ◽  
Long Qt Syndrome ◽  
Long Qt ◽  
Qt Syndrome

scholarly journals KCNQ1/KCNE1 K+ Channels Associated with Long QT Syndrome are Expressed in Early Stage Human Embryonic Stem Cell-Derived Cardiomyocytes

2010 ◽  
Vol 98 (3) ◽  
pp. 335a
Author(s):  
Cecile Terrenoire ◽  
George K. Wang ◽  
Eric Adler ◽  
Aaron D. Kaplan ◽  
Kevin J. Sampson ◽  
...  
Keyword(s):  
Stem Cell ◽  
Embryonic Stem Cell ◽  
Long Qt Syndrome ◽  
Human Embryonic Stem Cell ◽  
Early Stage ◽  
Embryonic Stem ◽  
Long Qt ◽  
K Channels ◽  
Qt Syndrome

scholarly journals Cardiac Small Conductance Calcium-Activated K Channels Maintain Repolarization Reserve in a Pharmacological Model of Type 3 Long QT Syndrome

2016 ◽  
Vol 110 (3) ◽  
pp. 447a-448a
Author(s):  
Jum Suk Ko ◽  
Dechun Yin ◽  
Thomas H. Everett ◽  
Zhenhui Chen ◽  
Michael Rubart ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Long Qt ◽  
K Channels ◽  
Repolarization Reserve ◽  
Qt Syndrome ◽  
Type 3 ◽  
Pharmacological Model ◽  
Small Conductance

Cellular mechanisms underlying the increased disease severity seen for patients with long QT syndrome caused by compound mutations in KCNQ1

2014 ◽  
Vol 462 (1) ◽  
pp. 133-142 ◽  
Author(s):  
Stephen C. Harmer ◽  
Jagdeep S. Mohal ◽  
Alice A. Royal ◽  
William J. McKenna ◽  
Pier D. Lambiase ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Disease Severity ◽  
Long Qt Syndrome ◽  
Clinical Phenotype ◽  
Syndrome Type ◽  
Cellular Mechanisms ◽  
Long Qt ◽  
Mutant Channel ◽  
Qt Syndrome

Long QT syndrome type 1 compound mutations in KCNQ1 are associated with a severe clinical phenotype. We identify that the retention of compound mutant channel complexes in the endoplasmic reticulum may underlie why patients with this phenotype have an increased arrhythmic risk.

Characteristics of Ca2+ -activated K+ channels isolated from the left ventricle of a patient with idiopathic long QT syndrome

1993 ◽  
Vol 126 (5) ◽  
pp. 1134-1141 ◽  
Author(s):  
Philip C. Krause ◽  
David P. Rardon ◽  
William M. Miles ◽  
Lawrence S. Klein ◽  
Yousuf Mahomed ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Long Qt Syndrome ◽  
Long Qt ◽  
K Channels ◽  
Qt Syndrome

The Hereditary Long QT Syndrome

2010 ◽  
Vol 43 (2) ◽  
pp. 19
Author(s):  
PETER HULICK
Keyword(s):  
Long Qt Syndrome ◽  
Long Qt ◽  
Qt Syndrome
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