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Items: 1 to 20 of 22


Molecular pathogenesis of long QT syndrome type 2.

Smith JL, Anderson CL, Burgess DE, Elayi CS, January CT, Delisle BP.

J Arrhythm. 2016 Oct;32(5):373-380. Epub 2016 Jan 22. Review.


Cardiac Delayed Rectifier Potassium Channels in Health and Disease.

Chen L, Sampson KJ, Kass RS.

Card Electrophysiol Clin. 2016 Jun;8(2):307-22. doi: 10.1016/j.ccep.2016.01.004. Epub 2016 Apr 1. Review.


Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane.

Apaja PM, Foo B, Okiyoneda T, Valinsky WC, Barriere H, Atanasiu R, Ficker E, Lukacs GL, Shrier A.

Mol Biol Cell. 2013 Dec;24(24):3787-804. doi: 10.1091/mbc.E13-07-0417. Epub 2013 Oct 23.


Pharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum.

Smith JL, Reloj AR, Nataraj PS, Bartos DC, Schroder EA, Moss AJ, Ohno S, Horie M, Anderson CL, January CT, Delisle BP.

Am J Physiol Cell Physiol. 2013 Nov 1;305(9):C919-30. doi: 10.1152/ajpcell.00406.2012. Epub 2013 Jul 17.


Pharmacologic Approach to Defective Protein Trafficking in the E637K-hERG Mutant with PD-118057 and Thapsigargin.

Mao H, Lu X, Karush JM, Huang X, Yang X, Ba Y, Wang Y, Liu N, Zhou J, Lian J.

PLoS One. 2013 Jun 19;8(6):e65481. doi: 10.1371/journal.pone.0065481. Print 2013.


Inside job: ligand-receptor pharmacology beneath the plasma membrane.

Babcock JJ, Li M.

Acta Pharmacol Sin. 2013 Jul;34(7):859-69. doi: 10.1038/aps.2013.51. Epub 2013 May 20. Review.


The interaction of caveolin 3 protein with the potassium inward rectifier channel Kir2.1: physiology and pathology related to long qt syndrome 9 (LQT9).

Vaidyanathan R, Vega AL, Song C, Zhou Q, Tan BH, Berger S, Makielski JC, Eckhardt LL.

J Biol Chem. 2013 Jun 14;288(24):17472-80. doi: 10.1074/jbc.M112.435370. Epub 2013 May 2. Erratum in: J Biol Chem. 2013 Oct 4;288(40):28948. Tan, Bihua [corrected to Tan, Bi-Hua].


Sequence and structure-specific elements of HERG mRNA determine channel synthesis and trafficking efficiency.

Sroubek J, Krishnan Y, McDonald TV.

FASEB J. 2013 Aug;27(8):3039-53. doi: 10.1096/fj.12-227009. Epub 2013 Apr 22.


Effects of cellular, chemical, and pharmacological chaperones on the rescue of a trafficking-defective mutant of the ATP-binding cassette transporter proteins ABCB1/ABCB4.

Gautherot J, Durand-Schneider AM, Delautier D, Delaunay JL, Rada A, Gabillet J, Housset C, Maurice M, Aït-Slimane T.

J Biol Chem. 2012 Feb 10;287(7):5070-8. doi: 10.1074/jbc.M111.275438. Epub 2011 Dec 19.


Molecular and cellular effects of Tamm-Horsfall protein mutations and their rescue by chemical chaperones.

Ma L, Liu Y, El-Achkar TM, Wu XR.

J Biol Chem. 2012 Jan 6;287(2):1290-305. doi: 10.1074/jbc.M111.283036. Epub 2011 Nov 22.


Interaction between the cardiac rapidly (IKr) and slowly (IKs) activating delayed rectifier potassium channels revealed by low K+-induced hERG endocytic degradation.

Guo J, Wang T, Yang T, Xu J, Li W, Fridman MD, Fisher JT, Zhang S.

J Biol Chem. 2011 Oct 7;286(40):34664-74. doi: 10.1074/jbc.M111.253351. Epub 2011 Aug 15.


Trafficking-deficient hERG K⁺ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER.

Smith JL, McBride CM, Nataraj PS, Bartos DC, January CT, Delisle BP.

Am J Physiol Cell Physiol. 2011 Jul;301(1):C75-85. doi: 10.1152/ajpcell.00494.2010. Epub 2011 Apr 13.


Trafficking defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutation.

Mihic A, Chauhan VS, Gao X, Oudit GY, Tsushima RG.

PLoS One. 2011 Mar 31;6(3):e18273. doi: 10.1371/journal.pone.0018273.


Trafficking-competent KCNQ1 variably influences the function of HERG long QT alleles.

Hayashi K, Shuai W, Sakamoto Y, Higashida H, Yamagishi M, Kupershmidt S.

Heart Rhythm. 2010 Jul;7(7):973-80. doi: 10.1016/j.hrthm.2010.03.038. Epub 2010 Mar 27.


Hsp40 chaperones promote degradation of the HERG potassium channel.

Walker VE, Wong MJ, Atanasiu R, Hantouche C, Young JC, Shrier A.

J Biol Chem. 2010 Jan 29;285(5):3319-29. doi: 10.1074/jbc.M109.024000. Epub 2009 Nov 25.


Rescue of a trafficking defective human pacemaker channel via a novel mechanism: roles of Src, Fyn, and Yes tyrosine kinases.

Lin YC, Huang J, Kan H, Frisbee JC, Yu HG.

J Biol Chem. 2009 Oct 30;284(44):30433-40. doi: 10.1074/jbc.M109.039180. Epub 2009 Sep 11.


Small GTPase determinants for the Golgi processing and plasmalemmal expression of human ether-a-go-go related (hERG) K+ channels.

Delisle BP, Underkofler HA, Moungey BM, Slind JK, Kilby JA, Best JM, Foell JD, Balijepalli RC, Kamp TJ, January CT.

J Biol Chem. 2009 Jan 30;284(5):2844-53. doi: 10.1074/jbc.M807289200. Epub 2008 Nov 24.


Pharmacological approach to the treatment of long and short QT syndromes.

Patel C, Antzelevitch C.

Pharmacol Ther. 2008 Apr;118(1):138-51. doi: 10.1016/j.pharmthera.2008.02.001. Review.


Improved functional expression of recombinant human ether-a-go-go (hERG) K+ channels by cultivation at reduced temperature.

Chen MX, Sandow SL, Doceul V, Chen YH, Harper H, Hamilton B, Meadows HJ, Trezise DJ, Clare JJ.

BMC Biotechnol. 2007 Dec 20;7:93.


Disease-associated mutations affect intracellular traffic and paracellular Mg2+ transport function of Claudin-16.

Kausalya PJ, Amasheh S, Günzel D, Wurps H, Müller D, Fromm M, Hunziker W.

J Clin Invest. 2006 Apr;116(4):878-91. Epub 2006 Mar 9.

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