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■ Abbreviation / Long Form : LQT2 / long QT syndrome type 2

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Total Number of Papers: 58
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Abbreviation:   LQT2  (>> Co-occurring Abbreviation)
Long Form:   long QT syndrome type 2
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No. Year Title Co-occurring Abbreviation
2022 Establishment of human embryonic stem cell WAe009-A-74 carrying a Long QT syndrome mutation in KCNH2. hERG
2022 Establishment of human embryonic stem cell WAe009-A-88 carrying a long QT syndrome mutation in KCNH2. ---
2022 The Linkage Phase of the Polymorphism KCNH2-K897T Influences the Electrophysiological Phenotype in hiPSC Models of LQT2. hERG, hiPSC-CMs, hiPSCs, SNP
2021 Aborted Cardiac Arrest in LQT2 Related to Novel KCNH2 (hERG) Variant Identified in One Lithuanian Family. ECG, LQTS
2021 Derivation and characterization of two human induced pluripotent stem cell lines (NUIGi004-A) and (NUIGi012-A) from two patients with LQT2 disease. iPSC
2021 Establishment of a human-induced pluripotent stem cell line, KSCBi014-A, from a long QT syndrome type 2 patient harboring a KCNH2 mutation. iPSC
2021 Generation of three heterozygous KCNH2 mutation-carrying human induced pluripotent stem cell lines for modeling LQT2 syndrome. iPSC, PBMCs
2021 hERG-deficient human embryonic stem cell-derived cardiomyocytes for modelling QT prolongation. CM, hERG, hESC, MEA
2020 Generation and characterization of an induced pluripotent stem cell (iPSC) line (NUIGi003-A) from a long QT syndrome type 2 (LQT2) patient harbouring the KCNH2 c.2464G>A pathogenic variant. iPSC, LQTS
10  2020 High-throughput discovery of trafficking-deficient variants in the cardiac potassium channel KV11.1. ---
11  2020 hiPSC-Derived Cardiomyocyte Model of LQT2 Syndrome Derived from Asymptomatic and Symptomatic Mutation Carriers Reproduces Clinical Differences in Aggregates but Not in Single Cells. hiPSC-CM
12  2020 Outcome by Sex in Patients With Long QT Syndrome With an Implantable Cardioverter Defibrillator. ICD, LQT1, LQT3
13  2019 Mutation-specific peripheral and ER quality control of hERG channel cell-surface expression. ER, PAS, PM, QC
14  2019 Short-Long Heart Rate Variation Increases Dispersion of Action Potential Duration in Long QT Type 2 Transgenic Rabbit Model. CLs, LMC
15  2018 Comparison of automated interval measurements by widely used algorithms in digital electrocardiographs. ECG, LQT1
16  2018 Functional analysis of KCNH2 gene mutations of type 2 long QT syndrome in larval zebrafish using microscopy and electrocardiography. ECG, MO, WT
17  2018 NCX-Mediated Subcellular Ca2+ Dynamics Underlying Early Afterdepolarizations in LQT2 Cardiomyocytes. EADs
18  2018 Restoration of mutant hERG stability by inhibition of HDAC6. HDACs, hERG, TBA, TSA, WT
19  2018 Visualizing Mutation-Specific Differences in the Trafficking-Deficient Phenotype of Kv11.1 Proteins Linked to Long QT Syndrome Type 2. ER, QC
20  2017 (-)-Epicatechin rescues the As2 O3 -induced HERG K+ channel deficiency possibly through upregulating transcription factor SP1 expression. EPI, hERG
21  2017 Characteristics of electromechanical window in anesthetized rabbit models of short QT and long QT syndromes. EMW, SQTS, TdP
22  2017 Identification of a targeted and testable antiarrhythmic therapy for long-QT syndrome type 2 using a patient-specific cellular model. cFPD, iPSC-CMs, LUM
23  2017 Noninvasive quantification of blood potassium concentration from ECG in hemodialysis patients. ECG
24  2017 Nonsense-Mediated mRNA Decay of hERG Mutations in Long QT Syndrome. hERG, NMD, PTCs
25  2017 NS1643 enhances ionic currents in a G604S-WT hERG co-expression system associated with long QT syndrome 2. hERG
26  2017 Patients With Long-QT Syndrome Caused by Impaired hERG-Encoded Kv11.1 Potassium Channel Have Exaggerated Endocrine Pancreatic and Incretin Function Associated With Reactive Hypoglycemia. GIP, GLP-1
27  2017 The KCNH2-IVS9-28A/G mutation causes aberrant isoform expression and hERG trafficking defect in cardiomyocytes derived from patients affected by Long QT Syndrome type 2. iPSC-CMs
28  2016 Characterization of the novel mutant A78T-HERG from a long QT syndrome type 2 patient: Instability of the mutant protein and stabilization by heat shock factor 1. hERG, HS, HSFs, WT-HERG
29  2016 Experience with bisoprolol in long-QT1 and long-QT2 syndrome. ECG, LQT, LQT1
30  2016 Generation of human induced pluripotent stem cell line from a patient with a long QT syndrome type 2. ECACC
31  2016 hERG quality control and the long QT syndrome. CF, ER, hERG, PM, QC
32  2016 Time, frequency and information domain analysis of heart period and QT variability in asymptomatic long QT syndrome type 2 patients. ASYMP, HP, NMC
33  2014 Hyperphosphorylation of RyRs underlies triggered activity in transgenic rabbit model of LQT2 syndrome. EADs, hERG, RyR
34  2014 Identification and functional characterization of the human ether-a-go-go-related gene Q738X mutant associated with hereditary long QT syndrome type 2. hERG, WT
35  2014 Position of premature termination codons determines susceptibility of hERG mutations to nonsense-mediated mRNA decay in long QT syndrome. NMD, PTC
36  2014 Progesterone modulates SERCA2a expression and function in rabbit cardiomyocytes. SERCA2a
37  2013 LQT2 nonsense mutations generate trafficking defective NH2-terminally truncated channels by the reinitiation of translation. hERG, NMD
38  2013 The generation of induced pluripotent stem cells from a patient with KCNH2 G603D, without LQT2 disease associated symptom. iPS
39  2013 The structural mechanism of KCNH-channel regulation by the eag domain. CNBHD, PAS
40  2012 Early LQT2 nonsense mutation generates N-terminally truncated hERG channels with altered gating properties by the reinitiation of translation. hERG, NMD
41  2012 Estradiol promotes sudden cardiac death in transgenic long QT type 2 rabbits while progesterone is protective. DHT, EST, PROG, PVT, SCD
42  2012 Trafficking-deficient G572R-hERG and E637K-hERG activate stress and clearance pathways in endoplasmic reticulum. hERG, UPR
43  2011 Functional characterization of the LQT2-causing mutation R582C and the associated voltage-dependent fluorescence signal. hERG, WT
44  2011 Nonsense-mediated mRNA decay caused by a frameshift mutation in a large kindred of type 2 long QT syndrome. NMD
45  2011 Protein kinase A activity at the endoplasmic reticulum surface is responsible for augmentation of human ether-a-go-go-related gene product (HERG). ER, hERG, PKA
46  2011 Trafficking-deficient hERG K⁺ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER. ER, ERGIC, hERG, WT-hERG
47  2010 Calcium oscillations and T-wave lability precede ventricular arrhythmias in acquired long QT type 2. APs, CaT, EADs, ECG, NCX, TdP, TWL
48  2010 Electrophysiological studies of transgenic long QT type 1 and type 2 rabbits reveal genotype-specific differences in ventricular refractoriness and His conduction. AV, LMC, LQT1, VERP
49  2010 Inhibition of nonsense-mediated mRNA decay by antisense morpholino oligonucleotides restores functional expression of hERG nonsense and frameshift mutations in long-QT syndrome. hERG, NMD
50  2010 Multiple splicing defects caused by hERG splice site mutation 2592+1G>A associated with long QT syndrome. hERG
51  2010 Properties of WT and mutant hERG K(+) channels expressed in neonatal mouse cardiomyocytes. HEK, WT
52  2010 Trigger-specific risk factors and response to therapy in long QT syndrome type 2. HR
53  2007 Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome. hERG
54  2005 Identification of the cyclic-nucleotide-binding domain as a conserved determinant of ion-channel cell-surface localization. CNBD, HERG
55  2005 Novel mutation in the Per-Arnt-Sim domain of KCNH2 causes a malignant form of long-QT syndrome. PAS
56  2004 Defective assembly and trafficking of mutant HERG channels with C-terminal truncations in long QT syndrome. hERG
57  2003 A new oral therapy for long QT syndrome: long-term oral potassium improves repolarization in patients with HERG mutations. Kr, LQTS
58  2001 The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations. hERG