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Pharmacological activation of IKr in models of long QT Type 2 risks overcorrection of repolarization.

Perry MD, Ng CA, Mangala MM, Ng TYM, Hines AD, Liang W, Xu MJO, Hill AP, Vandenberg JI.

Cardiovasc Res. 2019 Sep 13. pii: cvz247. doi: 10.1093/cvr/cvz247. [Epub ahead of print]


High-throughput phenotyping of heteromeric human ether-à-go-go-related gene potassium channel variants can discriminate pathogenic from rare benign variants.

Ng CA, Perry MD, Liang W, Smith NJ, Foo B, Shrier A, Lukacs GL, Hill AP, Vandenberg JI.

Heart Rhythm. 2019 Sep 23. pii: S1547-5271(19)30853-7. doi: 10.1016/j.hrthm.2019.09.020. [Epub ahead of print]


Protocol-Dependent Differences in IC50 Values Measured in Human Ether-Á-Go-Go-Related Gene Assays Occur in a Predictable Way and Can Be Used to Quantify State Preference of Drug Binding.

Lee W, Windley MJ, Perry MD, Vandenberg JI, Hill AP.

Mol Pharmacol. 2019 May;95(5):537-550. doi: 10.1124/mol.118.115220. Epub 2019 Feb 15.


Experimentally Validated Pharmacoinformatics Approach to Predict hERG Inhibition Potential of New Chemical Entities.

Munawar S, Windley MJ, Tse EG, Todd MH, Hill AP, Vandenberg JI, Jabeen I.

Front Pharmacol. 2018 Sep 19;9:1035. doi: 10.3389/fphar.2018.01035. eCollection 2018.


The Temperature Dependence of Kinetics Associated with Drug Block of hERG Channels Is Compound-Specific and an Important Factor for Proarrhythmic Risk Prediction.

Windley MJ, Lee W, Vandenberg JI, Hill AP.

Mol Pharmacol. 2018 Jul;94(1):760-769. doi: 10.1124/mol.117.111534. Epub 2018 May 4.


Sinusoidal voltage protocols for rapid characterisation of ion channel kinetics.

Beattie KA, Hill AP, Bardenet R, Cui Y, Vandenberg JI, Gavaghan DJ, de Boer TP, Mirams GR.

J Physiol. 2018 May 15;596(10):1813-1828. doi: 10.1113/JP275733. Epub 2018 Apr 17.


Never at rest: insights into the conformational dynamics of ion channels from cryo-electron microscopy.

Lau C, Hunter MJ, Stewart A, Perozo E, Vandenberg JI.

J Physiol. 2018 Apr 1;596(7):1107-1119. doi: 10.1113/JP274888. Epub 2018 Mar 5. Review.


In Vitro and In Silico Risk Assessment in Acquired Long QT Syndrome: The Devil Is in the Details.

Lee W, Windley MJ, Vandenberg JI, Hill AP.

Front Physiol. 2017 Nov 16;8:934. doi: 10.3389/fphys.2017.00934. eCollection 2017. Review.


Recent advances in understanding and prevention of sudden cardiac death.

Vandenberg JI, Perry MD, Hill AP.

F1000Res. 2017 Aug 31;6:1614. doi: 10.12688/f1000research.11855.1. eCollection 2017. Review.


Towards a Structural View of Drug Binding to hERG K+ Channels.

Vandenberg JI, Perozo E, Allen TW.

Trends Pharmacol Sci. 2017 Oct;38(10):899-907. doi: 10.1016/ Epub 2017 Jul 12. Review.


The S1 helix critically regulates the finely tuned gating of Kv11.1 channels.

Phan K, Ng CA, David E, Shishmarev D, Kuchel PW, Vandenberg JI, Perry MD.

J Biol Chem. 2017 May 5;292(18):7688-7705. doi: 10.1074/jbc.M117.779298. Epub 2017 Mar 9.


Measuring kinetics and potency of hERG block for CiPA.

Windley MJ, Abi-Gerges N, Fermini B, Hancox JC, Vandenberg JI, Hill AP.

J Pharmacol Toxicol Methods. 2017 Sep;87:99-107. doi: 10.1016/j.vascn.2017.02.017. Epub 2017 Feb 10.


Potassium channels in the heart: structure, function and regulation.

Grandi E, Sanguinetti MC, Bartos DC, Bers DM, Chen-Izu Y, Chiamvimonvat N, Colecraft HM, Delisle BP, Heijman J, Navedo MF, Noskov S, Proenza C, Vandenberg JI, Yarov-Yarovoy V.

J Physiol. 2017 Apr 1;595(7):2209-2228. doi: 10.1113/JP272864. Epub 2016 Nov 13. Review.


Potassium currents in the heart: functional roles in repolarization, arrhythmia and therapeutics.

Chiamvimonvat N, Chen-Izu Y, Clancy CE, Deschenes I, Dobrev D, Heijman J, Izu L, Qu Z, Ripplinger CM, Vandenberg JI, Weiss JN, Koren G, Banyasz T, Grandi E, Sanguinetti MC, Bers DM, Nerbonne JM.

J Physiol. 2017 Apr 1;595(7):2229-2252. doi: 10.1113/JP272883. Epub 2017 Jan 5. Review.


TECRL: connecting sequence to consequence for a new sudden cardiac death gene.

Perry MD, Vandenberg JI.

EMBO Mol Med. 2016 Dec 1;8(12):1364-1365. doi: 10.15252/emmm.201606967. Print 2016 Dec. No abstract available.


Convergence of models of human ventricular myocyte electrophysiology after global optimization to recapitulate clinical long QT phenotypes.

Mann SA, Imtiaz M, Winbo A, Rydberg A, Perry MD, Couderc JP, Polonsky B, McNitt S, Zareba W, Hill AP, Vandenberg JI.

J Mol Cell Cardiol. 2016 Nov;100:25-34. doi: 10.1016/j.yjmcc.2016.09.011. Epub 2016 Sep 20.


T-wave morphology can distinguish healthy controls from LQTS patients.

Immanuel SA, Sadrieh A, Baumert M, Couderc JP, Zareba W, Hill AP, Vandenberg JI.

Physiol Meas. 2016 Sep;37(9):1456-73. doi: 10.1088/0967-3334/37/9/1456. Epub 2016 Aug 11.


Tyrosine Residues from the S4-S5 Linker of Kv11.1 Channels Are Critical for Slow Deactivation.

Ng CA, Gravel AE, Perry MD, Arnold AA, Marcotte I, Vandenberg JI.

J Biol Chem. 2016 Aug 12;291(33):17293-302. doi: 10.1074/jbc.M116.729392. Epub 2016 Jun 17.


Heritability of ECG Biomarkers in the Netherlands Twin Registry Measured from Holter ECGs.

Hodkinson EC, Neijts M, Sadrieh A, Imtiaz MS, Baumert M, Subbiah RN, Hayward CS, Boomsma D, Willemsen G, Vandenberg JI, Hill AP, De Geus E.

Front Physiol. 2016 Apr 29;7:154. doi: 10.3389/fphys.2016.00154. eCollection 2016.


Temperature Effects on Kinetics of KV11.1 Drug Block Have Important Consequences for In Silico Proarrhythmic Risk Prediction.

Windley MJ, Mann SA, Vandenberg JI, Hill AP.

Mol Pharmacol. 2016 Jul;90(1):1-11. doi: 10.1124/mol.115.103127. Epub 2016 May 12.


An 'alternans' way to quantify arrhythmogenic substrates.

Vandenberg JI, Hill AP.

J Physiol. 2016 May 1;594(9):2375-6. doi: 10.1113/JP271838. No abstract available.


The two-pore domain potassium channel, TWIK-1, has a role in the regulation of heart rate and atrial size.

Christensen AH, Chatelain FC, Huttner IG, Olesen MS, Soka M, Feliciangeli S, Horvat C, Santiago CF, Vandenberg JI, Schmitt N, Olesen SP, Lesage F, Fatkin D.

J Mol Cell Cardiol. 2016 Aug;97:24-35. doi: 10.1016/j.yjmcc.2016.04.006. Epub 2016 Apr 19.


Computational cardiology and risk stratification for sudden cardiac death: one of the grand challenges for cardiology in the 21st century.

Hill AP, Perry MD, Abi-Gerges N, Couderc JP, Fermini B, Hancox JC, Knollmann BC, Mirams GR, Skinner J, Zareba W, Vandenberg JI.

J Physiol. 2016 Dec 1;594(23):6893-6908. doi: 10.1113/JP272015. Epub 2016 Jun 9.


Rescue of protein expression defects may not be enough to abolish the pro-arrhythmic phenotype of long QT type 2 mutations.

Perry MD, Ng CA, Phan K, David E, Steer K, Hunter MJ, Mann SA, Imtiaz M, Hill AP, Ke Y, Vandenberg JI.

J Physiol. 2016 Jul 15;594(14):4031-49. doi: 10.1113/JP271805. Epub 2016 May 27.


In silico assessment of kinetics and state dependent binding properties of drugs causing acquired LQTS.

Lee W, Mann SA, Windley MJ, Imtiaz MS, Vandenberg JI, Hill AP.

Prog Biophys Mol Biol. 2016 Jan;120(1-3):89-99. doi: 10.1016/j.pbiomolbio.2015.12.005. Epub 2015 Dec 20.


Differential Response to Risperidone in Schizophrenia Patients by KCNH2 Genotype and Drug Metabolizer Status.

Heide J, Zhang F, Bigos KL, Mann SA, Carr VJ, Shannon Weickert C, Green MJ, Weinberger DR, Vandenberg JI.

Am J Psychiatry. 2016 Jan;173(1):53-9. doi: 10.1176/appi.ajp.2015.14050653. Epub 2015 Oct 20.


A New Perspective in the Field of Cardiac Safety Testing through the Comprehensive In Vitro Proarrhythmia Assay Paradigm.

Fermini B, Hancox JC, Abi-Gerges N, Bridgland-Taylor M, Chaudhary KW, Colatsky T, Correll K, Crumb W, Damiano B, Erdemli G, Gintant G, Imredy J, Koerner J, Kramer J, Levesque P, Li Z, Lindqvist A, Obejero-Paz CA, Rampe D, Sawada K, Strauss DG, Vandenberg JI.

J Biomol Screen. 2016 Jan;21(1):1-11. doi: 10.1177/1087057115594589. Epub 2015 Jul 13. Review.


Getting to the heart of hERG K(+) channel gating.

Perry MD, Ng CA, Mann SA, Sadrieh A, Imtiaz M, Hill AP, Vandenberg JI.

J Physiol. 2015 Jun 15;593(12):2575-85. doi: 10.1113/JP270095. Review.


Can many subunits make light work of ion channel inactivation?

Vandenberg JI, Perry MD.

J Physiol. 2014 Oct 15;592(20):4411-2. doi: 10.1113/jphysiol.2014.281568. No abstract available.


Multiscale cardiac modelling reveals the origins of notched T waves in long QT syndrome type 2.

Sadrieh A, Domanski L, Pitt-Francis J, Mann SA, Hodkinson EC, Ng CA, Perry MD, Taylor JA, Gavaghan D, Subbiah RN, Vandenberg JI, Hill AP.

Nat Commun. 2014 Sep 25;5:5069. doi: 10.1038/ncomms6069.


Multiple interactions between cytoplasmic domains regulate slow deactivation of Kv11.1 channels.

Ng CA, Phan K, Hill AP, Vandenberg JI, Perry MD.

J Biol Chem. 2014 Sep 12;289(37):25822-32. doi: 10.1074/jbc.M114.558379. Epub 2014 Jul 29.


The Romano-Ward syndrome--1964-2014: 50 years of progress.

Hodkinson EC, Hill AP, Vandenberg JI.

Ir Med J. 2014 Apr;107(4):122-4.


Role of the cytoplasmic N-terminal Cap and Per-Arnt-Sim (PAS) domain in trafficking and stabilization of Kv11.1 channels.

Ke Y, Hunter MJ, Ng CA, Perry MD, Vandenberg JI.

J Biol Chem. 2014 May 16;289(20):13782-91. doi: 10.1074/jbc.M113.531277. Epub 2014 Apr 2.


Kinetics of drug interaction with the Kv11.1 potassium channel.

Hill AP, Perrin MJ, Heide J, Campbell TJ, Mann SA, Vandenberg JI.

Mol Pharmacol. 2014 May;85(5):769-76. doi: 10.1124/mol.114.091835. Epub 2014 Feb 28.


Genetic variation in the two-pore domain potassium channel, TASK-1, may contribute to an atrial substrate for arrhythmogenesis.

Liang B, Soka M, Christensen AH, Olesen MS, Larsen AP, Knop FK, Wang F, Nielsen JB, Andersen MN, Humphreys D, Mann SA, Huttner IG, Vandenberg JI, Svendsen JH, Haunsø S, Preiss T, Seebohm G, Olesen SP, Schmitt N, Fatkin D.

J Mol Cell Cardiol. 2014 Feb;67:69-76. doi: 10.1016/j.yjmcc.2013.12.014. Epub 2013 Dec 27.


C-terminal β9-strand of the cyclic nucleotide-binding homology domain stabilizes activated states of Kv11.1 channels.

Ng CA, Ke Y, Perry MD, Tan PS, Hill AP, Vandenberg JI.

PLoS One. 2013 Oct 25;8(10):e77032. doi: 10.1371/journal.pone.0077032. eCollection 2013.


Hydrophobic interactions between the voltage sensor and pore mediate inactivation in Kv11.1 channels.

Perry MD, Wong S, Ng CA, Vandenberg JI.

J Gen Physiol. 2013 Sep;142(3):275-88. doi: 10.1085/jgp.201310975.


A transgenic zebrafish model of a human cardiac sodium channel mutation exhibits bradycardia, conduction-system abnormalities and early death.

Huttner IG, Trivedi G, Jacoby A, Mann SA, Vandenberg JI, Fatkin D.

J Mol Cell Cardiol. 2013 Aug;61:123-32. doi: 10.1016/j.yjmcc.2013.06.005. Epub 2013 Jun 19.


Trafficking defects in PAS domain mutant Kv11.1 channels: roles of reduced domain stability and altered domain-domain interactions.

Ke Y, Ng CA, Hunter MJ, Mann SA, Heide J, Hill AP, Vandenberg JI.

Biochem J. 2013 Aug 15;454(1):69-77. doi: 10.1042/BJ20130328.


Quantifying the origins of population variability in cardiac electrical activity through sensitivity analysis of the electrocardiogram.

Sadrieh A, Mann SA, Subbiah RN, Domanski L, Taylor JA, Vandenberg JI, Hill AP.

J Physiol. 2013 Sep 1;591(17):4207-22. doi: 10.1113/jphysiol.2013.251710. Epub 2013 Apr 3.


Pore helices play a dynamic role as integrators of domain motion during Kv11.1 channel inactivation gating.

Perry MD, Ng CA, Vandenberg JI.

J Biol Chem. 2013 Apr 19;288(16):11482-91. doi: 10.1074/jbc.M113.461442. Epub 2013 Mar 7.


From kinetics to imaging: an NMR odyssey--a festschrift symposium in honour of Philip William Kuchel.

Vandenberg JI, Conigrave A, King GF, Kirk K.

Eur Biophys J. 2013 Jan;42(1):1-2. doi: 10.1007/s00249-012-0883-8. No abstract available.


The schizophrenia-associated Kv11.1-3.1 isoform results in reduced current accumulation during repetitive brief depolarizations.

Heide J, Mann SA, Vandenberg JI.

PLoS One. 2012;7(9):e45624. doi: 10.1371/journal.pone.0045624. Epub 2012 Sep 24.


R222Q SCN5A mutation is associated with reversible ventricular ectopy and dilated cardiomyopathy.

Mann SA, Castro ML, Ohanian M, Guo G, Zodgekar P, Sheu A, Stockhammer K, Thompson T, Playford D, Subbiah R, Kuchar D, Aggarwal A, Vandenberg JI, Fatkin D.

J Am Coll Cardiol. 2012 Oct 16;60(16):1566-73. doi: 10.1016/j.jacc.2012.05.050. Epub 2012 Sep 19.


hERG K(+) channels: structure, function, and clinical significance.

Vandenberg JI, Perry MD, Perrin MJ, Mann SA, Ke Y, Hill AP.

Physiol Rev. 2012 Jul;92(3):1393-478. Review.


Voltage-sensing domain mode shift is coupled to the activation gate by the N-terminal tail of hERG channels.

Tan PS, Perry MD, Ng CA, Vandenberg JI, Hill AP.

J Gen Physiol. 2012 Sep;140(3):293-306. doi: 10.1085/jgp.201110761. Epub 2012 Aug 13.


Hodgkin and Huxley and the basis for electrical signalling: a remarkable legacy still going strong.

Vandenberg JI, Waxman SG.

J Physiol. 2012 Jun 1;590(11):2569-70. doi: 10.1113/jphysiol.2012.233411. No abstract available.


Insights into hERG K+ channel structure and function from NMR studies.

Ng CA, Torres AM, Pagès G, Kuchel PW, Vandenberg JI.

Eur Biophys J. 2013 Jan;42(1):71-9. doi: 10.1007/s00249-012-0808-6. Epub 2012 May 3. Review.


Epistatic effects of potassium channel variation on cardiac repolarization and atrial fibrillation risk.

Mann SA, Otway R, Guo G, Soka M, Karlsdotter L, Trivedi G, Ohanian M, Zodgekar P, Smith RA, Wouters MA, Subbiah R, Walker B, Kuchar D, Sanders P, Griffiths L, Vandenberg JI, Fatkin D.

J Am Coll Cardiol. 2012 Mar 13;59(11):1017-25. doi: 10.1016/j.jacc.2011.11.039.

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