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Volumn 9, Issue 1, 2010, Pages 404-412

Probing the metabolic aberrations underlying mutant huntingtin toxicity in yeast and assessing their degree of preservation in humans and mice

Author keywords

Disease models; Huntington's disease; Metabolism; Metabolomics; Neurodegenerative disease; Phenotypic profiling; Yeast

Indexed keywords

ACETIC ACID; ALANINE; BIOLOGICAL MARKER; GALACTOSE; GLUTAMINE; GLYCEROL; HISTIDINE; HUNTINGTIN; PROLINE; SUCCINIC ACID; THREONINE; TREHALOSE; VALINE;

EID: 73649095908     PISSN: 15353893     EISSN: None     Source Type: Journal    
DOI: 10.1021/pr900734g     Document Type: Article
Times cited : (22)

References (54)
  • 2
    • 33846225133 scopus 로고    scopus 로고
    • Huntington's disease
    • Walker, F. O. Huntington's disease. Lancet 2007, 369 (9557), 218-228.
    • (2007) Lancet , vol.369 , Issue.9557 , pp. 218-228
    • Walker, F.O.1
  • 3
    • 77349122826 scopus 로고    scopus 로고
    • CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches
    • DOI: 10.1002/ajmg.b.30992
    • Langbehn, D. R.; Hayden, M. R.; Paulsen, J. S. CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches. Am. J. Med. Genet., Part B 2009, DOI: 10.1002/ajmg.b.30992.
    • (2009) Am. J. Med. Genet., Part B
    • Langbehn, D.R.1    Hayden, M.R.2    Paulsen, J.S.3
  • 4
    • 37849030901 scopus 로고    scopus 로고
    • Polyglutamine diseases: Emerging concepts in pathogenesis and therapy
    • Shao, J.; Diamond, M. I. Polyglutamine diseases: emerging concepts in pathogenesis and therapy. Hum. Mol. Genet. 2007, 16 (R2), R115-123.
    • (2007) Hum. Mol. Genet , vol.16 , Issue.R2
    • Shao, J.1    Diamond, M.I.2
  • 7
    • 33847663446 scopus 로고    scopus 로고
    • Selective degeneration in YAC mouse models of Huntington disease
    • Van Raamsdonk, J. M.; Warby, S. C.; Hayden, M. R. Selective degeneration in YAC mouse models of Huntington disease. Brain Res. Bull. 2007, 72 (2-3), 124-131.
    • (2007) Brain Res. Bull , vol.72 , Issue.2-3 , pp. 124-131
    • Van Raamsdonk, J.M.1    Warby, S.C.2    Hayden, M.R.3
  • 9
    • 59649109467 scopus 로고    scopus 로고
    • Decreased BDNF levels are a major contributor to the embryonic phenotype of huntingtin knockdown zebrafish
    • Diekmann, H.; Anichtchik, O.; Fleming, A.; Futter, M.; Goldsmith, P.; Roach, A.; Rubinsztein, D. C. Decreased BDNF levels are a major contributor to the embryonic phenotype of huntingtin knockdown zebrafish. J. Neurosci. 2009, 29 (5), 1343-1349.
    • (2009) J. Neurosci , vol.29 , Issue.5 , pp. 1343-1349
    • Diekmann, H.1    Anichtchik, O.2    Fleming, A.3    Futter, M.4    Goldsmith, P.5    Roach, A.6    Rubinsztein, D.C.7
  • 11
    • 34548406422 scopus 로고    scopus 로고
    • Huntingtin-deficient zebrafish exhibit defects in iron utilization and development
    • Lumsden, A. L.; Henshall, T. L.; Dayan, S.; Lardelli, M. T.; Richards, R. I. Huntingtin-deficient zebrafish exhibit defects in iron utilization and development. Hum. Mol. Genet. 2007, 16 (16), 1905-1920.
    • (2007) Hum. Mol. Genet , vol.16 , Issue.16 , pp. 1905-1920
    • Lumsden, A.L.1    Henshall, T.L.2    Dayan, S.3    Lardelli, M.T.4    Richards, R.I.5
  • 12
    • 0032168160 scopus 로고    scopus 로고
    • Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons
    • Jackson, G. R.; Salecker, I.; Dong, X.; Yao, X.; Arnheim, N.; Faber, P. W.; MacDonald, M. E.; Zipursky, S. L. Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Neuron 1998, 21 (3), 633-642.
    • (1998) Neuron , vol.21 , Issue.3 , pp. 633-642
    • Jackson, G.R.1    Salecker, I.2    Dong, X.3    Yao, X.4    Arnheim, N.5    Faber, P.W.6    MacDonald, M.E.7    Zipursky, S.L.8
  • 13
    • 38349171781 scopus 로고    scopus 로고
    • Branco, J.; Al-Ramahi, I.; Ukani, L.; Perez, A. M.; Fernandez-Funez, P.; Rincon-Limas, D.; Botas, J. Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum. Mol. Genet. 2008, 17 (3), 376-390.
    • Branco, J.; Al-Ramahi, I.; Ukani, L.; Perez, A. M.; Fernandez-Funez, P.; Rincon-Limas, D.; Botas, J. Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum. Mol. Genet. 2008, 17 (3), 376-390.
  • 17
    • 0037168585 scopus 로고    scopus 로고
    • Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity
    • Faber, P. W.; Voisine, C.; King, D. C.; Bates, E. A.; Hart, A. C. Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity. Proc. Natl. Acad. Sci. U.S.A. 2002, 99 (26), 17131-17136.
    • (2002) Proc. Natl. Acad. Sci. U.S.A , vol.99 , Issue.26 , pp. 17131-17136
    • Faber, P.W.1    Voisine, C.2    King, D.C.3    Bates, E.A.4    Hart, A.C.5
  • 18
    • 0033524413 scopus 로고    scopus 로고
    • Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron
    • Faber, P. W.; Alter, J. R.; MacDonald, M. E.; Hart, A. C. Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc. Natl. Acad. Sci. U.S.A. 1999, 96 (1), 179-184.
    • (1999) Proc. Natl. Acad. Sci. U.S.A , vol.96 , Issue.1 , pp. 179-184
    • Faber, P.W.1    Alter, J.R.2    MacDonald, M.E.3    Hart, A.C.4
  • 22
    • 66749167799 scopus 로고    scopus 로고
    • Subramaniam, S.; Sixt, K. M.; Barrow, R.; Snyder, S. H. Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity. Science 2009, 324 (5932), 1327-1330.
    • Subramaniam, S.; Sixt, K. M.; Barrow, R.; Snyder, S. H. Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity. Science 2009, 324 (5932), 1327-1330.
  • 23
    • 0037053566 scopus 로고    scopus 로고
    • Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1
    • Meriin, A. B.; Zhang, X.; He, X.; Newnam, G. P.; Chernoff, Y. O.; Sherman, M. Y. Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1. J. Cell Biol. 2002, 157 (6), 997-1004.
    • (2002) J. Cell Biol , vol.157 , Issue.6 , pp. 997-1004
    • Meriin, A.B.1    Zhang, X.2    He, X.3    Newnam, G.P.4    Chernoff, Y.O.5    Sherman, M.Y.6
  • 24
    • 67849092349 scopus 로고    scopus 로고
    • Exploiting Yeast Genetics to Inform Therapeutic Strategies for Huntington's Disease
    • Stagljar, I, Ed, Humana Press: New York
    • Giorgini, F.; Muchowski, P. J., Exploiting Yeast Genetics to Inform Therapeutic Strategies for Huntington's Disease. In Yeast Functional Genomics and Proteomics; Stagljar, I., Ed.; Humana Press: New York, 2009; Vol. 548, pp 161-174.
    • (2009) Yeast Functional Genomics and Proteomics , vol.548 , pp. 161-174
    • Giorgini, F.1    Muchowski, P.J.2
  • 25
    • 0034652127 scopus 로고    scopus 로고
    • Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins
    • Krobitsch, S.; Lindquist, S. Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins. Proc. Natl. Acad. Sci. U.S.A. 2000, 97 (4), 1589-1594.
    • (2000) Proc. Natl. Acad. Sci. U.S.A , vol.97 , Issue.4 , pp. 1589-1594
    • Krobitsch, S.1    Lindquist, S.2
  • 26
    • 60549103857 scopus 로고    scopus 로고
    • The R6 lines of transgenic mice: A model for screening new therapies for Huntington's disease
    • Gil, J. M.; Rego, A. C. The R6 lines of transgenic mice: a model for screening new therapies for Huntington's disease. Brain Res. Rev. 2009, 59 (2), 410-431.
    • (2009) Brain Res. Rev , vol.59 , Issue.2 , pp. 410-431
    • Gil, J.M.1    Rego, A.C.2
  • 27
    • 26844498655 scopus 로고    scopus 로고
    • The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies
    • Li, J. Y.; Popovic, N.; Brundin, P. The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies. NeuroRx 2005, 2 (3), 447-464.
    • (2005) NeuroRx , vol.2 , Issue.3 , pp. 447-464
    • Li, J.Y.1    Popovic, N.2    Brundin, P.3
  • 28
    • 26944458863 scopus 로고    scopus 로고
    • Compounds blocking mutant huntingtin toxicity identified using a Huntington's disease neuronal cell model
    • Wang, W.; Duan, W.; Igarashi, S.; Morita, H.; Nakamura, M.; Ross, C. A. Compounds blocking mutant huntingtin toxicity identified using a Huntington's disease neuronal cell model. Neurobiol. Dis. 2005, 20 (2), 500-508.
    • (2005) Neurobiol. Dis , vol.20 , Issue.2 , pp. 500-508
    • Wang, W.1    Duan, W.2    Igarashi, S.3    Morita, H.4    Nakamura, M.5    Ross, C.A.6
  • 31
    • 33749009306 scopus 로고    scopus 로고
    • Yeast as a drug discovery platform in Huntington's and Parkinson's diseases
    • Outeiro, T. F.; Giorgini, F. Yeast as a drug discovery platform in Huntington's and Parkinson's diseases. Biotechnol. J. 2006, 1 (3), 258-269.
    • (2006) Biotechnol. J , vol.1 , Issue.3 , pp. 258-269
    • Outeiro, T.F.1    Giorgini, F.2
  • 32
    • 36749006111 scopus 로고    scopus 로고
    • Bioactivity profiling with parallel mass spectrometry reveals an assemblage of green tea metabolites affording protection against human huntingtin and α-synuclein toxicity
    • Williams, R. B.; Gutekunst, W. R.; Joyner, P. M.; Duan, W.; Li, Q.; Ross, C. A.; Williams, T. D.; Cichewicz, R. H. Bioactivity profiling with parallel mass spectrometry reveals an assemblage of green tea metabolites affording protection against human huntingtin and α-synuclein toxicity. J. Agric. Food Chem. 2007, 55 (23), 9450-9456.
    • (2007) J. Agric. Food Chem , vol.55 , Issue.23 , pp. 9450-9456
    • Williams, R.B.1    Gutekunst, W.R.2    Joyner, P.M.3    Duan, W.4    Li, Q.5    Ross, C.A.6    Williams, T.D.7    Cichewicz, R.H.8
  • 34
    • 67349196013 scopus 로고    scopus 로고
    • Metabonomic characterization of the 3-nitropropionic acid rat model of Huntington's disease
    • Tsang, T. M.; Haselden, J. N.; Holmes, E. Metabonomic characterization of the 3-nitropropionic acid rat model of Huntington's disease. Neurochem. Res. 2009, 34 (7), 1261-1271.
    • (2009) Neurochem. Res , vol.34 , Issue.7 , pp. 1261-1271
    • Tsang, T.M.1    Haselden, J.N.2    Holmes, E.3
  • 35
    • 0026960116 scopus 로고
    • Correspondence analysis in medical research
    • Greenacre, M. Correspondence analysis in medical research. Stat. Methods Med. Res. 1992, 1 (1), 97-117.
    • (1992) Stat. Methods Med. Res , vol.1 , Issue.1 , pp. 97-117
    • Greenacre, M.1
  • 36
    • 34249879981 scopus 로고    scopus 로고
    • 2nd ed, Chapman & Hall/CRC Taylor & Francis Group: Boca Raton, FL
    • Greenacre, M., Correspondence analysis in practice, 2nd ed.; Chapman & Hall/CRC Taylor & Francis Group: Boca Raton, FL, 2007.
    • (2007) Correspondence analysis in practice
    • Greenacre, M.1
  • 37
    • 0017367349 scopus 로고
    • Correspondence analysis of HLA gene frequency data from 124 population samples
    • Greenacre, M. J.; Degos, L. Correspondence analysis of HLA gene frequency data from 124 population samples. Am. J. Hum. Genet. 1977, 1 (1), 60-75.
    • (1977) Am. J. Hum. Genet , vol.1 , Issue.1 , pp. 60-75
    • Greenacre, M.J.1    Degos, L.2
  • 38
    • 33745700438 scopus 로고    scopus 로고
    • Targeted profiling: Quantitative analysis of 1H NMR metabolomics data
    • Weljie, A. M.; Newton, J.; Mercier, P.; Carlson, E.; Slupsky, C. M. Targeted profiling: quantitative analysis of 1H NMR metabolomics data. Anal. Chem. 2006, 78 (13), 4430-4442.
    • (2006) Anal. Chem , vol.78 , Issue.13 , pp. 4430-4442
    • Weljie, A.M.1    Newton, J.2    Mercier, P.3    Carlson, E.4    Slupsky, C.M.5
  • 39
    • 28644433087 scopus 로고    scopus 로고
    • Normal huntingtin function: An alternative approach to Huntington's disease
    • Cattaneo, E.; Zuccato, C.; Tartari, M. Normal huntingtin function: an alternative approach to Huntington's disease. Nat. Rev. Neurosci. 2005, 6 (12), 919-930.
    • (2005) Nat. Rev. Neurosci , vol.6 , Issue.12 , pp. 919-930
    • Cattaneo, E.1    Zuccato, C.2    Tartari, M.3
  • 40
    • 0027272037 scopus 로고
    • CSF and serum metabolic profile of patients with Huntington's chorea: A study by high resolution proton NMR spectroscopy and HPLC
    • Nicoli, F.; Vion-Dury, J.; Maloteaux, J. M.; Delwaide, C.; Confort-Gouny, S.; Sciaky, M.; Cozzone, P. J. CSF and serum metabolic profile of patients with Huntington's chorea: a study by high resolution proton NMR spectroscopy and HPLC. Neurosci. Lett. 1993, 154 (1-2), 47-51.
    • (1993) Neurosci. Lett , vol.154 , Issue.1-2 , pp. 47-51
    • Nicoli, F.1    Vion-Dury, J.2    Maloteaux, J.M.3    Delwaide, C.4    Confort-Gouny, S.5    Sciaky, M.6    Cozzone, P.J.7
  • 41
    • 11144291556 scopus 로고    scopus 로고
    • 1H-MRS profiles of presymptomatic and early manifest Huntington's disease
    • 1H-MRS profiles of presymptomatic and early manifest Huntington's disease. Brain Res. 2005, 1031 (1), 82-89.
    • (2005) Brain Res , vol.1031 , Issue.1 , pp. 82-89
    • Reynolds, N.C.1    Prost, R.W.2    Mark, L.P.3
  • 42
    • 0029943742 scopus 로고    scopus 로고
    • Proton magnetic resonance spectroscopy in Huntington's disease: Evidence in favour of the glutamate excitotoxic theory
    • Taylor-Robinson, S. D.; Weeks, R. A.; Bryant, D. J.; Sargentoni, J.; Marcus, C. D.; Harding, A. E.; Brooks, D. J. Proton magnetic resonance spectroscopy in Huntington's disease: evidence in favour of the glutamate excitotoxic theory. Mov. Disord. 1996, 11 (2), 167-173.
    • (1996) Mov. Disord , vol.11 , Issue.2 , pp. 167-173
    • Taylor-Robinson, S.D.1    Weeks, R.A.2    Bryant, D.J.3    Sargentoni, J.4    Marcus, C.D.5    Harding, A.E.6    Brooks, D.J.7
  • 44
    • 26844550967 scopus 로고    scopus 로고
    • Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease
    • Jenkins, B. G.; Andreassen, O. A.; Dedeoglu, A.; Leavitt, B.; Hayden, M.; Borchelt, D.; Ross, C. A.; Ferrante, R. J.; Beal, M. F. Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease. J. Neurochem. 2005, 95 (2), 553-562.
    • (2005) J. Neurochem , vol.95 , Issue.2 , pp. 553-562
    • Jenkins, B.G.1    Andreassen, O.A.2    Dedeoglu, A.3    Leavitt, B.4    Hayden, M.5    Borchelt, D.6    Ross, C.A.7    Ferrante, R.J.8    Beal, M.F.9
  • 45
    • 0034006477 scopus 로고    scopus 로고
    • Nonlinear decrease over time in N-acetyl aspartate levels in the absence of neuronal loss and increases in glutamine and glucose in transgenic Huntington's disease mice
    • Jenkins, B. G.; Klivenyi, P.; Kustermann, E.; Andreassen, O. A.; Ferrante, R. J.; Rosen, B. R.; Beal, M. F. Nonlinear decrease over time in N-acetyl aspartate levels in the absence of neuronal loss and increases in glutamine and glucose in transgenic Huntington's disease mice. J. Neurochem. 2000, 74 (5), 2108-2119.
    • (2000) J. Neurochem , vol.74 , Issue.5 , pp. 2108-2119
    • Jenkins, B.G.1    Klivenyi, P.2    Kustermann, E.3    Andreassen, O.A.4    Ferrante, R.J.5    Rosen, B.R.6    Beal, M.F.7
  • 46
    • 33846970909 scopus 로고    scopus 로고
    • Neurochemical changes in Huntington R6/2 mouse striatum detected by in vivo 1H NMR spectroscopy
    • Tkac, I.; Dubinsky, J. M.; Keene, C. D.; Gruetter, R.; Low, W. C. Neurochemical changes in Huntington R6/2 mouse striatum detected by in vivo 1H NMR spectroscopy. J. Neurochem. 2007, 100 (5), 1397-1406.
    • (2007) J. Neurochem , vol.100 , Issue.5 , pp. 1397-1406
    • Tkac, I.1    Dubinsky, J.M.2    Keene, C.D.3    Gruetter, R.4    Low, W.C.5
  • 48
    • 57649121825 scopus 로고    scopus 로고
    • Metabolomics: A global biochemical approach to the study of central nervous system diseases
    • Kaddurah-Daouk, R.; Krishnan, K. R. R. Metabolomics: a global biochemical approach to the study of central nervous system diseases. Neuropsychopharmacology 2008, 34 (1), 173-186.
    • (2008) Neuropsychopharmacology , vol.34 , Issue.1 , pp. 173-186
    • Kaddurah-Daouk, R.1    Krishnan, K.R.R.2
  • 50
    • 72249116728 scopus 로고    scopus 로고
    • NMR metabolomics and drug discovery
    • DOI: 10.1002/mrc.2461
    • Powers, R. NMR metabolomics and drug discovery. Magn. Reson. Chem. 2009, DOI: 10.1002/mrc.2461.
    • (2009) Magn. Reson. Chem
    • Powers, R.1
  • 51
    • 14644442276 scopus 로고    scopus 로고
    • Discovery and development of biomarkers of neurological disease
    • Dunckley, T.; Coon, K. D.; Stephan, D. A. Discovery and development of biomarkers of neurological disease. Drug Discovery Today 2005, 10 (5), 326-334.
    • (2005) Drug Discovery Today , vol.10 , Issue.5 , pp. 326-334
    • Dunckley, T.1    Coon, K.D.2    Stephan, D.A.3
  • 52
    • 33748475897 scopus 로고    scopus 로고
    • Review: On the analysis and interpretation of correlations in metabolomic data
    • Steuer, R. Review: On the analysis and interpretation of correlations in metabolomic data. Briefings Bioinf. 2006, 7 (2), 151-158.
    • (2006) Briefings Bioinf , vol.7 , Issue.2 , pp. 151-158
    • Steuer, R.1
  • 53
    • 0027480960 scopus 로고    scopus 로고
    • MacDonald, M. E, Ambrose, C. M, Duyao, M. P, Myers, R. H, Lin, C, Srinidhi, L, Barnes, G, Taylor, S. A, James, M, Groot, N, MacFarlane, H, Jenkins, B, Anderson, M. A, Wexler, N. S, Gusella, J. F, Bates, G. P, Baxendale, S, Hummerich, H, Kirby, S, North, M, Youngman, S, Mott, R, Zehetner, G, Sedlacek, Z, Poustka, A, Frischauf, A.-M, Lehrach, H, Buckler, A. J, Church, D, Doucette-Stamm, L, O'Donovan, M. C, Riba-Ramirez, L, Shah, M, Stanton, V. P, Strobel, S. A, Draths, K. M, Wales, J. L, Dervan, P, Housman, D. E, Altherr, M, Shiang, R, Thompson, L, Fielder, T, Wasmuth, J. J, Tagle, D, Valdes, J, Elmer, L, Allard, M, Castilla, L, Swaroop, M, Blanchard, K, Collins, F. S, Snell, R, Holloway, T, Gillespie, K, Datson, N, Shaw, D, Harper, P. S. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993, 72 6, 971-983
    • MacDonald, M. E.; Ambrose, C. M.; Duyao, M. P.; Myers, R. H.; Lin, C.; Srinidhi, L.; Barnes, G.; Taylor, S. A.; James, M.; Groot, N.; MacFarlane, H.; Jenkins, B.; Anderson, M. A.; Wexler, N. S.; Gusella, J. F.; Bates, G. P.; Baxendale, S.; Hummerich, H.; Kirby, S.; North, M.; Youngman, S.; Mott, R.; Zehetner, G.; Sedlacek, Z.; Poustka, A.; Frischauf, A.-M.; Lehrach, H.; Buckler, A. J.; Church, D.; Doucette-Stamm, L.; O'Donovan, M. C.; Riba-Ramirez, L.; Shah, M.; Stanton, V. P.; Strobel, S. A.; Draths, K. M.; Wales, J. L.; Dervan, P.; Housman, D. E.; Altherr, M.; Shiang, R.; Thompson, L.; Fielder, T.; Wasmuth, J. J.; Tagle, D.; Valdes, J.; Elmer, L.; Allard, M.; Castilla, L.; Swaroop, M.; Blanchard, K.; Collins, F. S.; Snell, R.; Holloway, T.; Gillespie, K.; Datson, N.; Shaw, D.; Harper, P. S. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993, 72 (6), 971-983.


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