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Items: 1 to 50 of 66

1.

Long QT syndrome KCNH2 mutation with sequential fetal and maternal sudden death.

Tuveng JM, Berling BM, Bunford G, Vanoye CG, Welch RC, Leren TP, George AL Jr, Rognum TO.

Forensic Sci Med Pathol. 2018 Jun 8. doi: 10.1007/s12024-018-9989-3. [Epub ahead of print]

PMID:
29881912
2.

Mechanisms of KCNQ1 channel dysfunction in long QT syndrome involving voltage sensor domain mutations.

Huang H, Kuenze G, Smith JA, Taylor KC, Duran AM, Hadziselimovic A, Meiler J, Vanoye CG, George AL Jr, Sanders CR.

Sci Adv. 2018 Mar 7;4(3):eaar2631. doi: 10.1126/sciadv.aar2631. eCollection 2018 Mar.

3.

Characterization of a KCNB1 variant associated with autism, intellectual disability, and epilepsy.

Calhoun JD, Vanoye CG, Kok F, George AL Jr, Kearney JA.

Neurol Genet. 2017 Dec 11;3(6):e198. doi: 10.1212/NXG.0000000000000198. eCollection 2017 Dec.

4.

Predicting the Functional Impact of KCNQ1 Variants of Unknown Significance.

Li B, Mendenhall JL, Kroncke BM, Taylor KC, Huang H, Smith DK, Vanoye CG, Blume JD, George AL Jr, Sanders CR, Meiler J.

Circ Cardiovasc Genet. 2017 Oct;10(5). pii: e001754. doi: 10.1161/CIRCGENETICS.117.001754.

PMID:
29021305
5.

Allelic Complexity in Long QT Syndrome: A Family-Case Study.

Zullo A, Frisso G, Detta N, Sarubbi B, Romeo E, Cordella A, Vanoye CG, Calabrò R, George AL, Salvatore F.

Int J Mol Sci. 2017 Jul 27;18(8). pii: E1633. doi: 10.3390/ijms18081633.

6.

Sodium channel NaV1.9 mutations associated with insensitivity to pain dampen neuronal excitability.

Huang J, Vanoye CG, Cutts A, Goldberg YP, Dib-Hajj SD, Cohen CJ, Waxman SG, George AL Jr.

J Clin Invest. 2017 Jun 30;127(7):2805-2814. doi: 10.1172/JCI92373. Epub 2017 May 22.

7.

SCN3A deficiency associated with increased seizure susceptibility.

Lamar T, Vanoye CG, Calhoun J, Wong JC, Dutton SBB, Jorge BS, Velinov M, Escayg A, Kearney JA.

Neurobiol Dis. 2017 Jun;102:38-48. doi: 10.1016/j.nbd.2017.02.006. Epub 2017 Feb 22.

8.

Structural basis for KCNE3 modulation of potassium recycling in epithelia.

Kroncke BM, Van Horn WD, Smith J, Kang C, Welch RC, Song Y, Nannemann DP, Taylor KC, Sisco NJ, George AL Jr, Meiler J, Vanoye CG, Sanders CR.

Sci Adv. 2016 Sep 9;2(9):e1501228. doi: 10.1126/sciadv.1501228. eCollection 2016 Sep.

9.

Use-Dependent Block of Human Cardiac Sodium Channels by GS967.

Potet F, Vanoye CG, George AL Jr.

Mol Pharmacol. 2016 Jul;90(1):52-60. doi: 10.1124/mol.116.103358. Epub 2016 May 2.

10.

Reactivation of Lysosomal Ca2+ Efflux Rescues Abnormal Lysosomal Storage in FIG4-Deficient Cells.

Zou J, Hu B, Arpag S, Yan Q, Hamilton A, Zeng YS, Vanoye CG, Li J.

J Neurosci. 2015 Apr 29;35(17):6801-12. doi: 10.1523/JNEUROSCI.4442-14.2015.

11.

Personalized biochemistry and biophysics.

Kroncke BM, Vanoye CG, Meiler J, George AL Jr, Sanders CR.

Biochemistry. 2015 Apr 28;54(16):2551-9. doi: 10.1021/acs.biochem.5b00189. Epub 2015 Apr 15.

12.

De novo KCNB1 mutations in epileptic encephalopathy.

Torkamani A, Bersell K, Jorge BS, Bjork RL Jr, Friedman JR, Bloss CS, Cohen J, Gupta S, Naidu S, Vanoye CG, George AL Jr, Kearney JA.

Ann Neurol. 2014 Oct;76(4):529-540. doi: 10.1002/ana.24263. Epub 2014 Sep 19.

13.

SCN10A/Nav1.8 modulation of peak and late sodium currents in patients with early onset atrial fibrillation.

Savio-Galimberti E, Weeke P, Muhammad R, Blair M, Ansari S, Short L, Atack TC, Kor K, Vanoye CG, Olesen MS, LuCamp, Yang T, George AL Jr, Roden DM, Darbar D.

Cardiovasc Res. 2014 Nov 1;104(2):355-63. doi: 10.1093/cvr/cvu170. Epub 2014 Jul 22.

14.

Antiepileptic activity of preferential inhibitors of persistent sodium current.

Anderson LL, Thompson CH, Hawkins NA, Nath RD, Petersohn AA, Rajamani S, Bush WS, Frankel WN, Vanoye CG, Kearney JA, George AL Jr.

Epilepsia. 2014 Aug;55(8):1274-83. doi: 10.1111/epi.12657. Epub 2014 May 23.

15.

Purification and structural study of the voltage-sensor domain of the human KCNQ1 potassium ion channel.

Peng D, Kim JH, Kroncke BM, Law CL, Xia Y, Droege KD, Van Horn WD, Vanoye CG, Sanders CR.

Biochemistry. 2014 Apr 1;53(12):2032-42. doi: 10.1021/bi500102w. Epub 2014 Mar 18.

16.

Strain- and age-dependent hippocampal neuron sodium currents correlate with epilepsy severity in Dravet syndrome mice.

Mistry AM, Thompson CH, Miller AR, Vanoye CG, George AL Jr, Kearney JA.

Neurobiol Dis. 2014 May;65:1-11. doi: 10.1016/j.nbd.2014.01.006. Epub 2014 Jan 14.

17.

Novel SCN3A variants associated with focal epilepsy in children.

Vanoye CG, Gurnett CA, Holland KD, George AL Jr, Kearney JA.

Neurobiol Dis. 2014 Feb;62:313-22. doi: 10.1016/j.nbd.2013.10.015. Epub 2013 Oct 21.

18.

Selective targeting of gain-of-function KCNQ1 mutations predisposing to atrial fibrillation.

Campbell CM, Campbell JD, Thompson CH, Galimberti ES, Darbar D, Vanoye CG, George AL Jr.

Circ Arrhythm Electrophysiol. 2013 Oct;6(5):960-6. doi: 10.1161/CIRCEP.113.000439. Epub 2013 Sep 4.

19.

Mechanism of sodium channel NaV1.9 potentiation by G-protein signaling.

Vanoye CG, Kunic JD, Ehring GR, George AL Jr.

J Gen Physiol. 2013 Feb;141(2):193-202. doi: 10.1085/jgp.201210919.

20.

Reconstitution of KCNE1 into lipid bilayers: comparing the structural, dynamic, and activity differences in micelle and vesicle environments.

Coey AT, Sahu ID, Gunasekera TS, Troxel KR, Hawn JM, Swartz MS, Wickenheiser MR, Reid RJ, Welch RC, Vanoye CG, Kang C, Sanders CR, Lorigan GA.

Biochemistry. 2011 Dec 20;50(50):10851-9. doi: 10.1021/bi2009294. Epub 2011 Nov 22.

21.

Voltage-gated potassium channel KCNV2 (Kv8.2) contributes to epilepsy susceptibility.

Jorge BS, Campbell CM, Miller AR, Rutter ED, Gurnett CA, Vanoye CG, George AL Jr, Kearney JA.

Proc Natl Acad Sci U S A. 2011 Mar 29;108(13):5443-8. doi: 10.1073/pnas.1017539108. Epub 2011 Mar 14.

22.

Working model for the structural basis for KCNE1 modulation of the KCNQ1 potassium channel.

Van Horn WD, Vanoye CG, Sanders CR.

Curr Opin Struct Biol. 2011 Apr;21(2):283-91. doi: 10.1016/j.sbi.2011.01.001. Epub 2011 Feb 4. Review.

23.

KCNE4 juxtamembrane region is required for interaction with calmodulin and for functional suppression of KCNQ1.

Ciampa EJ, Welch RC, Vanoye CG, George AL Jr.

J Biol Chem. 2011 Feb 11;286(6):4141-9. doi: 10.1074/jbc.M110.158865. Epub 2010 Nov 30.

24.

KCNQ1/KCNE1 assembly, co-translation not required.

Vanoye CG, Welch RC, Tian C, Sanders CR, George AL Jr.

Channels (Austin). 2010 Mar-Apr;4(2):108-14. Epub 2010 Mar 6.

25.

Functional delivery of a membrane protein into oocyte membranes using bicelles.

Kang C, Vanoye CG, Welch RC, Van Horn WD, Sanders CR.

Biochemistry. 2010 Feb 2;49(4):653-5. doi: 10.1021/bi902155t.

26.

Distinct subdomains of the KCNQ1 S6 segment determine channel modulation by different KCNE subunits.

Vanoye CG, Welch RC, Daniels MA, Manderfield LJ, Tapper AR, Sanders CR, George AL Jr.

J Gen Physiol. 2009 Sep;134(3):207-17. doi: 10.1085/jgp.200910234. Epub 2009 Aug 17.

27.

CLCNKB-T481S and essential hypertension in a Ghanaian population.

Sile S, Velez DR, Gillani NB, Narsia T, Moore JH, George AL Jr, Vanoye CG, Williams SM.

J Hypertens. 2009 Feb;27(2):298-304.

28.

KCNE4 domains required for inhibition of KCNQ1.

Manderfield LJ, Daniels MA, Vanoye CG, George AL Jr.

J Physiol. 2009 Jan 15;587(2):303-14. doi: 10.1113/jphysiol.2008.161281. Epub 2008 Nov 24.

29.

Exaggerated Mg2+ inhibition of Kir2.1 as a consequence of reduced PIP2 sensitivity in Andersen syndrome.

Ballester LY, Vanoye CG, George AL Jr.

Channels (Austin). 2007 May-Jun;1(3):209-17. Epub 2007 Jul 20.

PMID:
18690034
30.

Structure of KCNE1 and implications for how it modulates the KCNQ1 potassium channel.

Kang C, Tian C, Sönnichsen FD, Smith JA, Meiler J, George AL Jr, Vanoye CG, Kim HJ, Sanders CR.

Biochemistry. 2008 Aug 5;47(31):7999-8006. doi: 10.1021/bi800875q. Epub 2008 Jul 9.

31.

Cardiac potassium channel dysfunction in sudden infant death syndrome.

Rhodes TE, Abraham RL, Welch RC, Vanoye CG, Crotti L, Arnestad M, Insolia R, Pedrazzini M, Ferrandi C, Vege A, Rognum T, Roden DM, Schwartz PJ, George AL Jr.

J Mol Cell Cardiol. 2008 Mar;44(3):571-81. doi: 10.1016/j.yjmcc.2007.11.015. Epub 2007 Dec 7.

32.

Structural models for the KCNQ1 voltage-gated potassium channel.

Smith JA, Vanoye CG, George AL Jr, Meiler J, Sanders CR.

Biochemistry. 2007 Dec 11;46(49):14141-52. Epub 2007 Nov 14.

33.

Functional BSND variants in essential hypertension.

Sile S, Gillani NB, Velez DR, Vanoye CG, Yu C, Byrne LM, Gainer JV, Brown NJ, Williams SM, George AL Jr.

Am J Hypertens. 2007 Nov;20(11):1176-1182.

PMID:
17954364
34.

Preparation, functional characterization, and NMR studies of human KCNE1, a voltage-gated potassium channel accessory subunit associated with deafness and long QT syndrome.

Tian C, Vanoye CG, Kang C, Welch RC, Kim HJ, George AL Jr, Sanders CR.

Biochemistry. 2007 Oct 16;46(41):11459-72. Epub 2007 Sep 25.

35.

Molecular physiology of renal ClC chloride channels/transporters.

Sile S, Vanoye CG, George AL Jr.

Curr Opin Nephrol Hypertens. 2006 Sep;15(5):511-6. Review.

PMID:
16914964
36.

Trafficking-competent and trafficking-defective KCNJ2 mutations in Andersen syndrome.

Ballester LY, Benson DW, Wong B, Law IH, Mathews KD, Vanoye CG, George AL Jr.

Hum Mutat. 2006 Apr;27(4):388.

PMID:
16541386
37.

Single-channel properties of human NaV1.1 and mechanism of channel dysfunction in SCN1A-associated epilepsy.

Vanoye CG, Lossin C, Rhodes TH, George AL Jr.

J Gen Physiol. 2006 Jan;127(1):1-14.

38.

Sodium channel dysfunction in intractable childhood epilepsy with generalized tonic-clonic seizures.

Rhodes TH, Vanoye CG, Ohmori I, Ogiwara I, Yamakawa K, George AL Jr.

J Physiol. 2005 Dec 1;569(Pt 2):433-45. Epub 2005 Oct 6.

39.

Polymorphic ventricular tachycardia and KCNJ2 mutations.

Chun TU, Epstein MR, Dick M 2nd, Andelfinger G, Ballester L, Vanoye CG, George AL Jr, Benson DW.

Heart Rhythm. 2004 Jul;1(2):235-41.

PMID:
15851159
40.

Expression of multiple KCNE genes in human heart may enable variable modulation of I(Ks).

Lundquist AL, Manderfield LJ, Vanoye CG, Rogers CS, Donahue BS, Chang PA, Drinkwater DC, Murray KT, George AL Jr.

J Mol Cell Cardiol. 2005 Feb;38(2):277-87. Epub 2005 Jan 20.

PMID:
15698834
41.

Noninactivating voltage-gated sodium channels in severe myoclonic epilepsy of infancy.

Rhodes TH, Lossin C, Vanoye CG, Wang DW, George AL Jr.

Proc Natl Acad Sci U S A. 2004 Jul 27;101(30):11147-52. Epub 2004 Jul 19.

42.

Epilepsy-associated dysfunction in the voltage-gated neuronal sodium channel SCN1A.

Lossin C, Rhodes TH, Desai RR, Vanoye CG, Wang D, Carniciu S, Devinsky O, George AL Jr.

J Neurosci. 2003 Dec 10;23(36):11289-95.

43.

Functional repair of a mutant chloride channel using a trans-splicing ribozyme.

Rogers CS, Vanoye CG, Sullenger BA, George AL Jr.

J Clin Invest. 2002 Dec;110(12):1783-9.

44.

KCNJ2 mutation results in Andersen syndrome with sex-specific cardiac and skeletal muscle phenotypes.

Andelfinger G, Tapper AR, Welch RC, Vanoye CG, George AL Jr, Benson DW.

Am J Hum Genet. 2002 Sep;71(3):663-8. Epub 2002 Jul 29.

45.

Molecular basis of an inherited epilepsy.

Lossin C, Wang DW, Rhodes TH, Vanoye CG, George AL Jr.

Neuron. 2002 Jun 13;34(6):877-84.

46.

The carboxyl termini of K(ATP) channels bind nucleotides.

Vanoye CG, MacGregor GG, Dong K, Tang L, Buschmann AS, Hall AE, Lu M, Giebisch G, Hebert SC.

J Biol Chem. 2002 Jun 28;277(26):23260-70. Epub 2002 Apr 15.

47.
48.

Nucleotides and phospholipids compete for binding to the C terminus of KATP channels.

MacGregor GG, Dong K, Vanoye CG, Tang L, Giebisch G, Hebert SC.

Proc Natl Acad Sci U S A. 2002 Mar 5;99(5):2726-31.

49.

An amino acid triplet in the NH2 terminus of rat ROMK1 determines interaction with SUR2B.

Dong K, Xu J, Vanoye CG, Welch R, MacGregor GG, Giebisch G, Hebert SC.

J Biol Chem. 2001 Nov 23;276(47):44347-53. Epub 2001 Sep 20.

50.

From stones to bones: the biology of ClC chloride channels.

George AL Jr, Bianchi L, Link EM, Vanoye CG.

Curr Biol. 2001 Aug 7;11(15):R620-8. Review.

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