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

American Journal of Physiology - Cell Physiology

Volumn 301, Issue 1, 2011, Pages

  Smith, Jennifer L ^a   McBride, Christie M ^a   Nataraj, Parvathi S ^a   Bartos, Daniel C ^a   January, Craig T ^b   Delisle, Brian P ^a  

^a UNIVERSITY OF KENTUCKY   (United States)

^b UNIVERSITY OF WISCONSIN MADISON   (United States)

Author keywords

Endoplasmic reticulum; Ion channel; Quality control; Trafficking

Indexed keywords

1 [2 (6 METHYL 2 PYRIDYL)ETHYL] 4 (4 METHYLSULFONYLAMINOBENZOYL)PIPERIDINE; CALNEXIN; ENDOPLASMIC RETICULUM GOLGI INTERMEDIATE COMPARTMENT PROTEIN 53; LYSYLASPARTYLGLUTAMYLLEUCINE; NOCODAZOLE; POLYPEPTIDE; POTASSIUM CHANNEL HERG; UNCLASSIFIED DRUG;

ARTICLE; BIOACCUMULATION; CELLULAR DISTRIBUTION; CONTROLLED STUDY; DEPOLYMERIZATION; EMBRYO; ENDOPLASMIC RETICULUM; GOLGI COMPLEX; HUMAN; HUMAN CELL; LONG QT SYNDROME; LONG QT SYNDROME TYPE 2; MICROTUBULE; MISSENSE MUTATION; POTASSIUM TRANSPORT; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN GLYCOSYLATION; PROTEIN LOCALIZATION; QUALITY CONTROL; REGULATORY MECHANISM; TEMPERATURE SENSITIVITY; WILD TYPE;

ANTI-ARRHYTHMIA AGENTS; BLOTTING, WESTERN; ENDOPLASMIC RETICULUM; ETHER-A-GO-GO POTASSIUM CHANNELS; FLUORESCENT ANTIBODY TECHNIQUE; GLYCOSYLATION; GOLGI APPARATUS; GREEN FLUORESCENT PROTEINS; HEK293 CELLS; HUMANS; LONG QT SYNDROME; MICROTUBULES; MUTATION; MYOCYTES, CARDIAC; NOCODAZOLE; PATCH-CLAMP TECHNIQUES; PIPERIDINES; PROTEIN TRANSPORT; PYRIDINES; TUBULIN MODULATORS;

VESICULAR STOMATITIS VIRUS;

EID: 79959853596     PISSN: 03636143     EISSN: 15221563     Source Type: Journal    
DOI: 10.1152/ajpcell.00494.2010     Document Type: Article

References (33)

1. Anderson CL, Delisle BP, Anson BD, Kilby JA, Will ML, Tester DJ, Gong Q, Zhou Z, Ackerman MJ, January CT. Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism. Circulation 113: 365-373, 2006.
2. Appenzeller-Herzog C, Hauri HP. The ER-Golgi intermediate compartment (ERGIC): in search of its identity and function. J Cell Sci 1: 2173-2183, 2006.
3. Bolte S, Cordelieres FP. A guided tour into subcellular colocalization analysis in light microscopy. J Microsc 224: 213-232, 2006.
4. Cole NB, Sciaky N, Marotta A, Song J, Lippencott-Schwartz J. Golgi dispersal during microtubule disruption: regeneration of Golgi stacks at peripheral endoplasmic reticulum exit sites. Mol Biol Cell 7: 631-650, 1996a.
5. + channels by cultivation at reduced temperature. BMC Biotechnol 20: 93, 2007.
6. Costes SV, Daelemans D, Cho EH, Dobbin Z, Pavlakis G, Lockett S. Automatic and quantitative measurement of protein-protein colocalization in live cells. Biophys J 86: 3993-4003, 2004.
7. Delisle BP, Anderson CL, Balijepalli RC, Anson BD, Kamp TJ, January CT. Thapsigargin selectively rescues the trafficking defective LQT2 channels G601S and F805C. J Biol Chem 278: 35749-35754, 2003.
8. + channels. J Biol Chem 284: 2844-2853, 2009.0
9. Delisle BP, Slind JK, Kilby JA, Anderson CL, Anson BD, Balijepalli RC, Tester DJ, Ackerman MJ, Kamp TJ, January CT. Intragenic suppression of trafficking-defective KCNH2 channels associated with long QT syndrome. Mol Pharm 68: 233-240, 2005.
10. Delisle BP, Anson BD, Rajamani S, January CT. Biology of cardiac arrhythmias: ion channel protein trafficking. Circ Res 11: 1418-1428, 2004.
11. Ellgaard L, Helenius A. Quality control in the endop lasmic reticulum. Nat Rev Mol Cell Biol 4: 181-191, 2003.
12. Ficker E, Obejero-Paz CA, Zhao S, Brown AM. The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations. J Biol Chem 277: 4989-4998, 2002.
13. Gong Q, Anderson CL, January CT, Zhou Z. Role of glycosylation in cell surface expression and stability of HERG potassium channels. Am J Physiol Heart Circ Physiol 283: H77-H84, 2002.
14. Gong Q, Jones MA, Zhou Z. Mechanisms of pharmacological rescue of trafficking-defective hERG mutant channels in human long QT syndrome. J Biol Chem 281: 4069-4074, 2006.
15. Hammond C, Helenius A. Quality control in the secretory pathway: retention of a misfolded viral membrane glycoprotein involves cycling between the ER, intermediate compartment, and Golgi apparatus. J Cell Biol 126: 41-52, 1994.
16. Helenius A, Aebi M. Intracellular functions of N-linked glycans. Science 291: 2364-2369, 2001.
17. Kamhi-Nesher Shenkman MS, Tolchinsky S, Fromm SV, Ehrlich R, Lederkremer GZ. A novel quality control compartment derived from the endoplasmic reticulum. Mol Biol Cell 6: 1711-1723, 2001.
18. Lippincott-Schwartz J, Donaldson JG, Schweizer A, Berger EG, Hauri HP, Yuan LC, Klausner RD. Microtubule-dependent retrograde transport of proteins into the ER in the presence of brefeldin A suggests an ER recycling pathway. Cell 60: 821-836, 1990.
19. Mezzacasa A, Helenius A. The transitional ER defines a boundary for quality control in the secretion of tsO45 VSV glycoprotein. Traffic 3: 833-849, 2002.
20. Petrecca K, Atanasiu R, Akhavan A, Shrier A. N-linked glycosylation sites determine HERG channel surface membrane expression. J Physiol 15: 41-48, 1999.
21. Presley JF, Cole NB, Schroer TA, Hirschberg K, Zaal KJ, Lippincott-Schwartz J. ER-to-Golgi transport visualized in living cells. Nature 389: 81-85, 1997.
22. Sanguinetti MC, Jiang C, Curran ME, Keating MT. A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel. Cell 81: 299-307, 1995.
23. Scheel J, Matteoni R, Ludwig T, Hoflack B, Kreis TE. Microtubule depolymerization inhibits transport of cathepsin D from the Golgi apparatus to lysosomes. J Cell Sci 96: 711-720, 1990.
24. Schweizer A, Fransen JA, Matter K, Kreis TE, Ginsel L, Hauri HP. Identification of an intermediate compartment involved in protein transport from endoplasmic reticulum to Golgi apparatus. Eur J Cell Biol 53: 185-196, 1990.
25. Spiro MJ, Spiro RG. Release of polymerase oligosaccharides from vesticular stomatitis virus G protein during endoplasmic reticulum-associated degradation. Glycobiology 11: 803-811, 2001.
26. Spiliotis ET, Pentcheva T, Edidin M. Probing for membrane domains in the endoplasmic reticulum: retention and degradation of unassembled MHC class I molecules. Mol Biol Cell 13: 1566-1581, 2002.
27. Storrie B, White J, Röttger S, Stelzer EH, Suganuma T, Nilsson T. Recycling of golgi-resident glycosyltransferases through the ER reveals a novel pathway and provides an explanation for nocodazole-induced Golgi scattering. J Cell Biol 143: 1505-1521, 1998.
28. Thyberg J, Moskalewski S. Role of microtubules in the organization of the Golgi complex. Exp Cell Res 246: 263-279, 1999.
29. Trudeau MC, Warmke JW, Ganetzky B, Robertson GA. HERG, a human inward rectifier in the voltage-gated potassium channel family. Science 269: 92-95, 1995.
30. Warmke JW, Ganetzky B. A family of potassium channel genes related to eag in Drosophila and mammals. Proc Natl Acad Sci USA 91: 3438-3442, 1994.
31. Zhou Z, Gong Q, Epstein ML, January CT. HERG channel dysfunction in human long QT syndrome. J Biol Chem 273: 21061-21066, 1998.
32. Zhou Z, Gong Q, Ye B, Fan Z, Makielski JC, Robertson GA, January CT. Properties of HERG channels stably expressed in HEK 293 cells studied at physiological temperature. Biophys J 74: 230-241, 1998.
33. Zhou ZGQ, January CT. Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects. J Biol Chem 274: 31123-31126, 1999.