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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
Chemical Compound Review

glutamine     (2S)-2-amino-4-aminocarbonyl- butanoic acid

Synonyms: Cebrogen, Glavamin, Saforis, Stimulina, Nutrestore, ...
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Disease relevance of glutamine


Psychiatry related information on glutamine


High impact information on glutamine


Chemical compound and disease context of glutamine


Biological context of glutamine


Anatomical context of glutamine


Associations of glutamine with other chemical compounds


Gene context of glutamine


Analytical, diagnostic and therapeutic context of glutamine


  1. A critical role for amino-terminal glutamine/asparagine repeats in the formation and propagation of a yeast prion. DePace, A.H., Santoso, A., Hillner, P., Weissman, J.S. Cell (1998) [Pubmed]
  2. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Martindale, D., Hackam, A., Wieczorek, A., Ellerby, L., Wellington, C., McCutcheon, K., Singaraja, R., Kazemi-Esfarjani, P., Devon, R., Kim, S.U., Bredesen, D.E., Tufaro, F., Hayden, M.R. Nat. Genet. (1998) [Pubmed]
  3. Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Imbert, G., Saudou, F., Yvert, G., Devys, D., Trottier, Y., Garnier, J.M., Weber, C., Mandel, J.L., Cancel, G., Abbas, N., Dürr, A., Didierjean, O., Stevanin, G., Agid, Y., Brice, A. Nat. Genet. (1996) [Pubmed]
  4. Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel. Zhuchenko, O., Bailey, J., Bonnen, P., Ashizawa, T., Stockton, D.W., Amos, C., Dobyns, W.B., Subramony, S.H., Zoghbi, H.Y., Lee, C.C. Nat. Genet. (1997) [Pubmed]
  5. Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Katsuno, M., Adachi, H., Doyu, M., Minamiyama, M., Sang, C., Kobayashi, Y., Inukai, A., Sobue, G. Nat. Med. (2003) [Pubmed]
  6. Glutamine: mode of action in critical illness. Wischmeyer, P.E. Crit. Care Med. (2007) [Pubmed]
  7. Exogenous glutamine: the clinical evidence. Bongers, T., Griffiths, R.D., McArdle, A. Crit. Care Med. (2007) [Pubmed]
  8. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G.P., Davies, S.W., Lehrach, H., Wanker, E.E. Cell (1997) [Pubmed]
  9. Human neurodegenerative disease modeling using Drosophila. Bonini, N.M., Fortini, M.E. Annu. Rev. Neurosci. (2003) [Pubmed]
  10. Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Karpuj, M.V., Becher, M.W., Springer, J.E., Chabas, D., Youssef, S., Pedotti, R., Mitchell, D., Steinman, L. Nat. Med. (2002) [Pubmed]
  11. Lithium rescues toxicity of aggregate-prone proteins in Drosophila by perturbing Wnt pathway. Berger, Z., Ttofi, E.K., Michel, C.H., Pasco, M.Y., Tenant, S., Rubinsztein, D.C., O'Kane, C.J. Hum. Mol. Genet. (2005) [Pubmed]
  12. No evidence for expanded polyglutamine sequences in bipolar disorder and schizophrenia. Jones, A.L., Middle, F., Guy, C., Spurlock, G., Cairns, N.J., McGuffin, P., Craddock, N., Owen, M., O'Donovan, M.C. Mol. Psychiatry (1997) [Pubmed]
  13. RORalpha-Mediated Purkinje Cell Development Determines Disease Severity in Adult SCA1 Mice. Serra, H.G., Duvick, L., Zu, T., Carlson, K., Stevens, S., Jorgensen, N., Lysholm, A., Burright, E., Zoghbi, H.Y., Clark, H.B., Andresen, J.M., Orr, H.T. Cell (2006) [Pubmed]
  14. Bidirectional expression of CUG and CAG expansion transcripts and intranuclear polyglutamine inclusions in spinocerebellar ataxia type 8. Moseley, M.L., Zu, T., Ikeda, Y., Gao, W., Mosemiller, A.K., Daughters, R.S., Chen, G., Weatherspoon, M.R., Clark, H.B., Ebner, T.J., Day, J.W., Ranum, L.P. Nat. Genet. (2006) [Pubmed]
  15. Neurodegeneration: a case of arrested development? La Spada, A.R. Cell (2006) [Pubmed]
  16. Polyglutamine expansion of huntingtin impairs its nuclear export. Cornett, J., Cao, F., Wang, C.E., Ross, C.A., Bates, G.P., Li, S.H., Li, X.J. Nat. Genet. (2005) [Pubmed]
  17. In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets. Zhai, W., Jeong, H., Cui, L., Krainc, D., Tjian, R. Cell (2005) [Pubmed]
  18. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Tanaka, M., Machida, Y., Niu, S., Ikeda, T., Jana, N.R., Doi, H., Kurosawa, M., Nekooki, M., Nukina, N. Nat. Med. (2004) [Pubmed]
  19. Clioquinol down-regulates mutant huntingtin expression in vitro and mitigates pathology in a Huntington's disease mouse model. Nguyen, T., Hamby, A., Massa, S.M. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  20. Neurotoxic protein expression reveals connections between the circadian clock and mating behavior in Drosophila. Kadener, S., Villella, A., Kula, E., Palm, K., Pyza, E., Botas, J., Hall, J.C., Rosbash, M. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  21. Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Katsuno, M., Sang, C., Adachi, H., Minamiyama, M., Waza, M., Tanaka, F., Doyu, M., Sobue, G. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  22. Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons. Parker, J.A., Arango, M., Abderrahmane, S., Lambert, E., Tourette, C., Catoire, H., Néri, C. Nat. Genet. (2005) [Pubmed]
  23. Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Huynh, D.P., Figueroa, K., Hoang, N., Pulst, S.M. Nat. Genet. (2000) [Pubmed]
  24. Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Igarashi, S., Koide, R., Shimohata, T., Yamada, M., Hayashi, Y., Takano, H., Date, H., Oyake, M., Sato, T., Sato, A., Egawa, S., Ikeuchi, T., Tanaka, H., Nakano, R., Tanaka, K., Hozumi, I., Inuzuka, T., Takahashi, H., Tsuji, S. Nat. Genet. (1998) [Pubmed]
  25. The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Tsuda, H., Jafar-Nejad, H., Patel, A.J., Sun, Y., Chen, H.K., Rose, M.F., Venken, K.J., Botas, J., Orr, H.T., Bellen, H.J., Zoghbi, H.Y. Cell (2005) [Pubmed]
  26. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Warrick, J.M., Paulson, H.L., Gray-Board, G.L., Bui, Q.T., Fischbeck, K.H., Pittman, R.N., Bonini, N.M. Cell (1998) [Pubmed]
  27. A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease. Giorgini, F., Guidetti, P., Nguyen, Q., Bennett, S.C., Muchowski, P.J. Nat. Genet. (2005) [Pubmed]
  28. Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Ikeda, H., Yamaguchi, M., Sugai, S., Aze, Y., Narumiya, S., Kakizuka, A. Nat. Genet. (1996) [Pubmed]
  29. A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila. Kazantsev, A., Walker, H.A., Slepko, N., Bear, J.E., Preisinger, E., Steffan, J.S., Zhu, Y.Z., Gertler, F.B., Housman, D.E., Marsh, J.L., Thompson, L.M. Nat. Genet. (2002) [Pubmed]
  30. Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy. Mhatre, A.N., Trifiro, M.A., Kaufman, M., Kazemi-Esfarjani, P., Figlewicz, D., Rouleau, G., Pinsky, L. Nat. Genet. (1993) [Pubmed]
  31. Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Sánchez, I., Mahlke, C., Yuan, J. Nature (2003) [Pubmed]
  32. 17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Waza, M., Adachi, H., Katsuno, M., Minamiyama, M., Sang, C., Tanaka, F., Inukai, A., Doyu, M., Sobue, G. Nat. Med. (2005) [Pubmed]
  33. Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Emamian, E.S., Kaytor, M.D., Duvick, L.A., Zu, T., Tousey, S.K., Zoghbi, H.Y., Clark, H.B., Orr, H.T. Neuron (2003) [Pubmed]
  34. Specific amino acids in the critically ill patient--exogenous glutamine/arginine: a common denominator? Vermeulen, M.A., van de Poll, M.C., Ligthart-Melis, G.C., Dejong, C.H., van den Tol, M.P., Boelens, P.G., van Leeuwen, P.A. Crit. Care Med. (2007) [Pubmed]
  35. Metabolism of glutamine and glutathione via gamma-glutamyltranspeptidase and glutamate transport in Helicobacter pylori: possible significance in the pathophysiology of the organism. Shibayama, K., Wachino, J., Arakawa, Y., Saidijam, M., Rutherford, N.G., Henderson, P.J. Mol. Microbiol. (2007) [Pubmed]
  36. Quantifying reductive carboxylation flux of glutamine to lipid in a brown adipocyte cell line. Yoo, H., Antoniewicz, M.R., Stephanopoulos, G., Kelleher, J.K. J. Biol. Chem. (2008) [Pubmed]
  37. Erythrocyte glutamine depletion, altered redox environment, and pulmonary hypertension in sickle cell disease. Morris, C.R., Suh, J.H., Hagar, W., Larkin, S., Bland, D.A., Steinberg, M.H., Vichinsky, E.P., Shigenaga, M., Ames, B., Kuypers, F.A., Klings, E.S. Blood (2008) [Pubmed]
  38. HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain. Kalchman, M.A., Koide, H.B., McCutcheon, K., Graham, R.K., Nichol, K., Nishiyama, K., Kazemi-Esfarjani, P., Lynn, F.C., Wellington, C., Metzler, M., Goldberg, Y.P., Kanazawa, I., Gietz, R.D., Hayden, M.R. Nat. Genet. (1997) [Pubmed]
  39. Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Gauthier, L.R., Charrin, B.C., Borrell-Pagès, M., Dompierre, J.P., Rangone, H., Cordelières, F.P., De Mey, J., MacDonald, M.E., Lessmann, V., Humbert, S., Saudou, F. Cell (2004) [Pubmed]
  40. Mutations in the polyglutamine binding protein 1 gene cause X-linked mental retardation. Kalscheuer, V.M., Freude, K., Musante, L., Jensen, L.R., Yntema, H.G., Gécz, J., Sefiani, A., Hoffmann, K., Moser, B., Haas, S., Gurok, U., Haesler, S., Aranda, B., Nshedjan, A., Tzschach, A., Hartmann, N., Roloff, T.C., Shoichet, S., Hagens, O., Tao, J., Van Bokhoven, H., Turner, G., Chelly, J., Moraine, C., Fryns, J.P., Nuber, U., Hoeltzenbein, M., Scharff, C., Scherthan, H., Lenzner, S., Hamel, B.C., Schweiger, S., Ropers, H.H. Nat. Genet. (2003) [Pubmed]
  41. Disruption of CREB function in brain leads to neurodegeneration. Mantamadiotis, T., Lemberger, T., Bleckmann, S.C., Kern, H., Kretz, O., Martin Villalba, A., Tronche, F., Kellendonk, C., Gau, D., Kapfhammer, J., Otto, C., Schmid, W., Schütz, G. Nat. Genet. (2002) [Pubmed]
  42. The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1. Matilla, A., Koshy, B.T., Cummings, C.J., Isobe, T., Orr, H.T., Zoghbi, H.Y. Nature (1997) [Pubmed]
  43. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Panov, A.V., Gutekunst, C.A., Leavitt, B.R., Hayden, M.R., Burke, J.R., Strittmatter, W.J., Greenamyre, J.T. Nat. Neurosci. (2002) [Pubmed]
  44. Silencing polyglutamine degeneration with RNAi. Bonini, N.M., La Spada, A.R. Neuron (2005) [Pubmed]
  45. Dying for a cause: invertebrate genetics takes on human neurodegeneration. Driscoll, M., Gerstbrein, B. Nat. Rev. Genet. (2003) [Pubmed]
  46. Histone deacetylase inhibitors reduce polyglutamine toxicity. McCampbell, A., Taye, A.A., Whitty, L., Penney, E., Steffan, J.S., Fischbeck, K.H. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
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