Baclofen, a GABAB receptor agonist, enhances ubiquitin-proteasome system functioning and neuronal survival in Huntington's disease model mice

Biochemical and Biophysical Research Communications

Volumn 443, Issue 2, 2014, Pages 706-711

  Kim, Woori ^a   Seo, Hyemyung ^a  

^a Hanyang University   (South Korea)

Author keywords

GABAB receptor agonist; Huntington's disease; Neuronal survival; Ubiquitin proteasome system

Indexed keywords

4 AMINOBUTYRIC ACID B RECEPTOR STIMULATING AGENT; 4 AMINOBUTYRIC ACID RECEPTOR; BACLOFEN; CHYMOTRYPSIN; HUNTINGTIN; PROTEASOME; UBIQUITIN; UBIQUITIN PROTEIN LIGASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR DISORDER; CAG REPEAT; CELL SURVIVAL; CONTROLLED STUDY; HUMAN; HUNTINGTON CHOREA; IN VITRO STUDY; IN VIVO STUDY; MALE; MOTOR COORDINATION; MOTOR DYSFUNCTION; MOUSE; MUTANT; NERVE CELL; NERVE CELL STIMULATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEGRADATION; ROTAROD TEST; WILD TYPE; ANIMAL; CORPUS STRIATUM; CYTOLOGY; DISEASE MODEL; DRUG EFFECT; GABA(B) RECEPTOR AGONIST; METABOLISM; NEURONAL SURVIVAL; PATHOLOGY; UBIQUITIN-PROTEASOME SYSTEM;

MUS; MUS MUSCULUS;

GABA(B) RECEPTOR AGONIST; HUNTINGTON'S DISEASE; NEURONAL SURVIVAL; UBIQUITIN-PROTEASOME SYSTEM;

ANIMALS; BACLOFEN; CELL SURVIVAL; CORPUS STRIATUM; DISEASE MODELS, ANIMAL; GABA-B RECEPTOR AGONISTS; GABAERGIC NEURONS; HUMANS; HUNTINGTON DISEASE; MALE; MICE; UBIQUITIN-PROTEIN LIGASE COMPLEXES;

EID: 84892444881     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2013.12.034     Document Type: Article

References (39)

1. H. Heinsen, M. Strik, M. Bauer, K. Luther, G. Ulmar, D. Gangnus, G. Jungkunz, W. Eisenmenger, and M. Gotz Cortical and striatal neurone number in Huntington's disease Acta Neuropathol. 88 1994 320 333
2. H.Y. Zoghbi, and H.T. Orr Glutamine repeats and neurodegeneration Annu. Rev. Neurosci. 23 2000 217 247
3. T.H.s.D.S. Group A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group Cell 72 1993 971 983
4. C.A. Gutekunst, S.H. Li, H. Yi, J.S. Mulroy, S. Kuemmerle, R. Jones, D. Rye, R.J. Ferrante, S.M. Hersch, and X.J. Li Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology J. Neurosci. 19 1999 2522 2534
5. D.C. Rubinsztein, A. Wyttenbach, and J. Rankin Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts? J. Med. Genet. 36 1999 265 270
6. C.A. Ross, and M.A. Poirier Protein aggregation and neurodegenerative disease Nat. Med. 10 2004 S10 S17
7. E.J. Slow, J. van Raamsdonk, D. Rogers, S.H. Coleman, R.K. Graham, Y. Deng, R. Oh, N. Bissada, S.M. Hossain, Y.Z. Yang, X.J. Li, E.M. Simpson, C.A. Gutekunst, B.R. Leavitt, and M.R. Hayden Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease Hum. Mol. Genet. 12 2003 1555 1567
8. S.W. Davies, M. Turmaine, B.A. Cozens, M. DiFiglia, A.H. Sharp, C.A. Ross, E. Scherzinger, E.E. Wanker, L. Mangiarini, and G.P. Bates Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation Cell 90 1997 537 548
9. L. Petrucelli, and T.M. Dawson Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system Ann. Med. 36 2004 315 320
10. E.J. Bennett, T.A. Shaler, B. Woodman, K.Y. Ryu, T.S. Zaitseva, C.H. Becker, G.P. Bates, H. Schulman, and R.R. Kopito Global changes to the ubiquitin system in Huntington's disease Nature 448 2007 704 708
11. R. de Pril, B. Hobo, P. van Tijn, R.A. Roos, F.W. van Leeuwen, and D.F. Fischer Modest proteasomal inhibition by aberrant ubiquitin exacerbates aggregate formation in a Huntington disease mouse model Mol. Cell Neurosci. 43 2010 281 286
12. H. Seo, K.C. Sonntag, and O. Isacson Generalized brain and skin proteasome inhibition in Huntington's disease Ann. Neurol. 56 2004 319 328
13. N.G. Bowery GABAB receptor: a site of therapeutic benefit Curr. Opin. Pharmacol. 6 2006 37 43
14. Z.J. Huang, and P. Scheiffele GABA and neuroligin signaling: linking synaptic activity and adhesion in inhibitory synapse development Curr. Opin. Neurobiol. 18 2008 77 83
15. R.L. Macdonald, and R.W. Olsen GABAA receptor channels Annu. Rev. Neurosci. 17 1994 569 602
16. D.D. Mott, and D.V. Lewis The pharmacology and function of central GABAB receptors Int. Rev. Neurobiol. 36 1994 97 223
17. A. Couve, S.J. Moss, and M.N. Pangalos GABAB receptors: a new paradigm in G protein signaling Mol. Cell Neurosci. 16 2000 296 312
18. T. Takahashi, Y. Kajikawa, and T. Tsujimoto G-Protein-coupled modulation of presynaptic calcium currents and transmitter release by a GABAB receptor J. Neurosci. 18 1998 3138 3146
19. C. Ladera, M. del Carmen Godino, M. Jose Cabanero, M. Torres, M. Watanabe, R. Lujan, and J. Sanchez-Prieto Pre-synaptic GABA receptors inhibit glutamate release through GIRK channels in rat cerebral cortex J. Neurochem. 107 2008 1506 1517
20. H.S. Yin, K. Chen, S. Kalpana, and J.C. Shih Differential effects of chronic amphetamine and baclofen administration on cAMP levels and phosphorylation of CREB in distinct brain regions of wild type and monoamine oxidase B-deficient mice Synapse 60 2006 573 584
21. J.M. Van Raamsdonk, J. Pearson, D.A. Rogers, N. Bissada, A.W. Vogl, M.R. Hayden, and B.R. Leavitt Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease Hum. Mol. Genet. 14 2005 1379 1392
22. N. Francia, F. Cirulli, F. Chiarotti, A. Antonelli, L. Aloe, and E. Alleva Spatial memory deficits in middle-aged mice correlate with lower exploratory activity and a subordinate status: role of hippocampal neurotrophins Eur. J. Neurosci. 23 2006 711 728
23. H. Seo, W. Kim, and O. Isacson Compensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients Hum. Mol. Genet. 17 2008 3144 3153
24. A. Bauer, K. Zilles, A. Matusch, C. Holzmann, O. Riess, and S. von Horsten Regional and subtype selective changes of neurotransmitter receptor density in a rat transgenic for the Huntington's disease mutation J. Neurochem. 94 2005 639 650
25. J.H. Cha, A.S. Frey, S.A. Alsdorf, J.A. Kerner, C.M. Kosinski, L. Mangiarini, J.B. Penney Jr., S.W. Davies, G.P. Bates, and A.B. Young Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease Philos. Trans. R. Soc. London, B 354 1999 981 989
26. J.H. Cha, C.M. Kosinski, J.A. Kerner, S.A. Alsdorf, L. Mangiarini, S.W. Davies, J.B. Penney, G.P. Bates, and A.B. Young Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene Proc. Natl. Acad. Sci. USA 95 1998 6480 6485
27. L.F. Nicholson, R.L. Faull, H.J. Waldvogel, and M. Dragunow GABA and GABAA receptor changes in the substantia nigra of the rat following quinolinic acid lesions in the striatum closely resemble Huntington's disease Neuroscience 66 1995 507 521
28. 1H NMR spectroscopy J. Proteome. Res. 5 2006 483 492
29. H. Kuroda Gamma-aminobutyric acid (GABA) in cerebrospinal fluid Acta Med. Okayama 37 1983 167 177
30. Y. Li, X. Li, P.J. Harvey, and D.J. Bennett Effects of baclofen on spinal reflexes and persistent inward currents in motoneurons of chronic spinal rats with spasticity J. Neurophysiol. 92 2004 2694 2703
31. M. Malcangio, and N.G. Bowery GABA and its receptors in the spinal cord Trends Pharmacol. Sci. 17 1996 457 462
32. D.R. Hill, and N.G. Bowery 3H-baclofen and 3H-GABA bind to bicuculline-insensitive GABA B sites in rat brain Nature 290 1981 149 152
33. L.K. Pacey, S.P. Heximer, and D.R. Hampson Increased GABA(B) receptor-mediated signaling reduces the susceptibility of fragile X knockout mice to audiogenic seizures Mol. Pharmacol. 76 2009 18 24
34. B.J. van Hilten, W.J. van de Beek, J.I. Hoff, J.H. Voormolen, and E.M. Delhaas Intrathecal baclofen for the treatment of dystonia in patients with reflex sympathetic dystrophy N. Engl. J. Med. 343 2000 625 630
35. S. Waelter, A. Boeddrich, R. Lurz, E. Scherzinger, G. Lueder, H. Lehrach, and E.E. Wanker Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation Mol. Biol. Cell 12 2001 1393 1407
36. R. Layfield, J. Lowe, and L. Bedford The ubiquitin-proteasome system and neurodegenerative disorders Essays Biochem. 41 2005 157 171
37. Y. Chai, S.L. Koppenhafer, S.J. Shoesmith, M.K. Perez, and H.L. Paulson Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro Hum. Mol. Genet. 8 1999 673 682
38. R. de Pril, D.F. Fischer, M.L. Maat-Schieman, B. Hobo, R.A. de Vos, E.R. Brunt, E.M. Hol, R.A. Roos, and F.W. van Leeuwen Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases Hum. Mol. Genet. 13 2004 1803 1813
39. L. Menalled, B.F. El-Khodor, M. Patry, M. Suarez-Farinas, S.J. Orenstein, B. Zahasky, C. Leahy, V. Wheeler, X.W. Yang, M. MacDonald, A.J. Morton, G. Bates, J. Leeds, L. Park, D. Howland, E. Signer, A. Tobin, and D. Brunner Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models Neurobiol. Dis. 35 2009 319 336