Potassium channel dysfunction and depolarized resting membrane potential in a cell model of SCA3

Experimental Neurology

Volumn 201, Issue 1, 2006, Pages 182-192

  Jeub, Monika ^a   Herbst, Martin ^b   Spauschus, Alexander ^c   Fleischer, Henrik ^a   Klockgether, Thomas ^a   Wuellner, Ullrich ^a   Evert, Bernd O ^a  

^a UNIVERSITY HOSPITAL BONN   (Germany)

^b MAX DELBRÜCK CENTER FOR MOLECULAR MEDICINE   (Germany)

^c UNIVERSITY OF HAMBURG   (Germany)

Author keywords

Electrophysiological dysfunction; Neuronal dysfunction; PC12; Polyglutamine; Potassium channel; Resting membrane potential; SCA3

Indexed keywords

ATAXIN 3; POTASSIUM CHANNEL;

ANIMAL CELL; ARTICLE; CELL DIFFERENTIATION; CELL MEMBRANE DEPOLARIZATION; CELL STRAIN; CELL ULTRASTRUCTURE; CELL VIABILITY; CONTROLLED STUDY; ELECTROPHYSIOLOGY; MACHADO JOSEPH DISEASE; MEMBRANE STEADY POTENTIAL; NERVE CELL NECROSIS; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; RAT;

ANALYSIS OF VARIANCE; ANIMALS; CELL DIFFERENTIATION; CELL PROLIFERATION; CELL SURVIVAL; DELAYED RECTIFIER POTASSIUM CHANNELS; DOXYCYCLINE; HUMANS; MACHADO-JOSEPH DISEASE; MEMBRANE POTENTIALS; MICROSCOPY, ELECTRON; MUTATION; NERVE GROWTH FACTOR; NERVE TISSUE PROTEINS; NEURONS; NUCLEAR PROTEINS; PC12 CELLS; RATS; REPRESSOR PROTEINS; TETRODOTOXIN; TIME FACTORS; TRANSFECTION;

EID: 33747085155     PISSN: 00144886     EISSN: 10902430     Source Type: Journal    
DOI: 10.1016/j.expneurol.2006.03.029     Document Type: Article

References (37)

1. Ariano M.A., Cepeda C., Calvert C.R., Flores-Hernandez J., Hernandez-Echeagaray E., Klapstein G.J., Chandler S.H., Aronin N., DiFiglia M., and Levine M.S. Striatal potassium channel dysfunction in Huntington's disease transgenic mice. J. Neurophysiol. 93 (2005) 2565-2574
2. Beal F. Energetics in the pathogenesis of neurodegenerative diseases. Trends Neurosci. 23 (2000) 298-304
3. Calabresi P.M., DeMurtas M., Pisani A., Stefani A., Sancesario G., Mercuri N.B., and Bernardi G. Vulnerability of medium spiny striatal neurons to glutamate: role of Na+/K+ ATPase. Eur. J. Neurosci. 7 (1995) 1674-1683
4. Chai Y., Koppenhafer S.L., Bonini N.M., and Paulson H.L. Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J. Neurosci. 19 (1999) 10338-10347
5. Clark H.B., Burright E.N., Yunis W.S., Larson S., Wilcox C., Hartman B., Matilla A., Zoghbi H.Y., and Orr H.T. Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. J. Neurosci. 17 (1997) 7385-7395
6. DiFiglia M., Sapp E., Chase K.O., Davies S.W., Bates G.P., Vonsattel J.P., and Aronin N. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277 (1997) 1990-1993
7. Evert B.O., Wullner U., Schulz J.B., Weller M., Groscurth P., Trottier Y., Brice A., and Klockgether T. High level expression of expanded full-length ataxin-3 in vitro causes cell death and formation of intranuclear inclusions in neuronal cells. Hum. Mol. Genet. 8 (1999) 1169-1176
8. Gossen M., and Bujard H. Tight control of gene expression in mammalian cells by tetracycline-responsive promoters. Proc. Natl. Acad. Sci. U. S. A. 89 (1992) 5547-5551
9. Hamill O.P., Marty A., Neher E., Sakmann B., and Sigworth F.J. Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches. Pflugers Arch. 391 (1981) 85-100
10. Hodgson J.G., Agopyan N., Gutekunst C.A., Leavitt B.R., Lepiane F., Singaraja R., Smith D.J., Bissada N., McCutcheon K., Nasir J., Jamot L., Li X.J., Stevens M.E., Rosemond E., Roder J.C., Phillips A.G., Rubin E.M., Hersch S.M., and Hayden M.R. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23 (1999) 181-192
11. Ikeda H., Yamaguchi M., Sugai S., Aze Y., Narumiya S., and Kakizuka A. Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nat. Genet. 13 (1996) 196-202
12. Inoue T., Lin X., Kohlmeier K.A., Orr H.T., Zoghbi H.Y., and Ross W.N. Calcium dynamics and electrophysiological properties of cerebellar Purkinje cells in SCA1 transgenic mice. J. Neurophysiol. 85 (2001) 1750-1760
13. Kim Y., Uhm D.Y., Shin J., and Chung S. Modulation of delayed rectifier potassium channel by protein kinase C zeta-containing signaling complex in pheochromocytoma cells. Neuroscience 125 (2004) 359-368
14. Klapstein G.J., Fisher R.S., Zanjani H., Cepeda C., Jokel E.S., Chesselet M.F., and Levine M.S. Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice. J. Neurophysiol. 86 (2001) 2667-2677
15. Laforet G.A., Sapp E., Chase K., McIntyre C., Boyce F.M., Campbell M., Cadigan B.A., Warzecki L., Tagle D.A., Reddy P.H., Cepeda C., Calvert C.R., Jokel E.S., Klapstein G.J., Ariano M.A., Levine M.S., DiFiglia M., and Aronin N. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J. Neurosci. 21 (2001) 9112-9123
16. Lorenzetti D., Watase K., Xu B., Matzuk M.M., Orr H.T., and Zoghbi H.Y. Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus. Hum. Mol. Genet. 9 (2000) 779-785
17. Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., Lawton M., Trottier Y., Lehrach H., Davies S.W., and Bates G.P. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87 (1996) 493-506
18. McCampbell A., Taylor J.P., Taye A.A., Robitschek J., Li M., Walcott J., Merry D., Chai Y., Paulson H., Sobue G., and Fischbeck K.H. CREB-binding protein sequestration by expanded polyglutamine. Hum. Mol. Genet. 9 (2000) 2197-2202
19. Murphy K.P., Carter R.J., Lione L.A., Mangiarini L., Mahal A., Bates G.P., Dunnett S.B., and Morton A.J. Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation. J. Neurosci. 20 (2000) 5115-5123
20. Nagaoka U., Kim K., Jana N.R., Doi H., Maruyama M., Mitsui K., Oyama F., and Nukina N. Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. J. Neurochem. 91 (2004) 57-68
21. Nucifora Jr. F.C., Sasaki M., Peters M.F., Huang H., Cooper J.K., Yamada M., Takahashi H., Tsuji S., Troncoso J., Dawson V.L., Dawson T.M., and Ross C.A. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 291 (2001) 2423-2428
22. 2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease. Arch. Biochem. Biophys. 410 (2003) 1-6
23. Paulson H.L., Perez M.K., Trottier Y., Trojanowski J.Q., Subramony S.H., Das S.S., Vig P., Mandel J.L., Fischbeck K.H., and Pittman R.N. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19 (1997) 333-344
24. Perez M.K., Paulson H.L., Pendse S.J., Saionz S.J., Bonini N.M., and Pittman R.N. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. J. Cell Biol. 143 (1998) 1457-1470
25. 2+ channel currents arising from expanded trinucleotide repeats in spinocerebellar ataxia type 6. J. Neurosci. 21 (2001) 9185-9193
26. Restituito S., Thompson R.M., Eliet J., Raike R.S., Riedl M., Charnet P., and Gomez C.M. The polyglutamine expansion in spinocerebellar ataxia type 6 causes a beta subunit-specific enhanced activation of P/Q-type calcium channels in Xenopus oocytes. J. Neurosci. 20 (2000) 6394-6403
27. Shelbourne P.F., Killeen N., Hevner R.F., Johnston H.M., Tecott L., Lewandowski M., Ennis M., Ramirez L., Li Z., Iannicola C., Littman D.R., and Myers R.M. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. Hum. Mol. Genet. 8 (1999) 763-774
28. Shimohata T., Nakajima T., Yamada M., Uchida C., Onodera O., Naruse S., Kimura T., Koide R., Nozaki K., Sano Y., Ishiguro H., Sakoe K., Ooshima T., Sato A., Ikeuchi T., Oyake M., Sato T., Aoyagi Y., Hozumi I., Nagatsu T., Takiyama Y., Nishizawa M., Goto J., Kanazawa I., Davidson I., Tanese N., Takahashi H., and Tsuji S. Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet. 26 (2000) 29-36
29. Simmons M.A., and Schneider C.R. Regulation of M-type potassium current by intracellular nucleotide phosphates. J. Neurosci. 18 (1998) 6254-6260
30. Skinner P.J., Koshy B.T., Cummings C.J., Klement I.A., Helin K., Servadio A., Zoghbi H.Y., and Orr H.T. Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures. Nature 389 (1997) 971-974
31. Toru S., Murakoshi T., Ishikawa K., Saegusa H., Fujigasaki H., Uchihara T., Nagayama S., Osanai M., Mizusawa H., and Tanabe T. Spinocerebellar ataxia type 6 mutation alters P-type calcium channel function. J. Biol. Chem. 275 (2000) 10893-10898
32. Warrick J.M., Morabito L.M., Bilen J., Gordesky-Gold B., Faust L.Z., Paulson H.L., and Bonini N.M. Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol. Cell 18 (2005) 37-48
33. Winklhofer M., Matthias K., Seifert G., Stocker M., Sewing S., Herget T., Steinhauser C., and Saaler-Reinhardt S. Analysis of phosphorylation-dependent modulation of Kv1.1 potassium channels. Neuropharmacology 44 (2003) 829-842
34. Yamamoto A., Lucas J.J., and Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101 (2000) 57-66
35. Yue S., Serra H.G., Zoghbi H.Y., and Orr H.T. The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract. Hum. Mol. Genet. 10 (2001) 25-30
36. Zhuchenko O., Bailey J., Bonnen P., Ashizawa T., Stockton D.W., Amos C., Dobyns W.B., Subramony S.H., Zoghbi H.Y., and Lee C.C. Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel. Nat. Genet. 15 (1997) 62-69
37. Zoghbi H.Y., and Orr H.T. Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci. 23 (2000) 217-247