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Volumn 23, Issue 8, 2008, Pages 484-493

Minocycline for the treatment of amyotrophic lateral sclerosis: Neuroprotector or neurotoxin? Reflections on another failure of translational medicine;Minociclina para el tratamiento de la esclerosis lateral amiotrófica: ¿Neuroprotectora o neurotóxica? Reflexiones sobre otro fracaso de la medicina traslacional

Author keywords

Amyotrophic lateral sclerosis; Clinical trial; Lou Gehrig's disease; Minocycline; Motor neuron disease; Neuroprotection; Neurotoxicity; Translational medicine

Indexed keywords

ACETYLSALICYLIC ACID; ANTIINFLAMMATORY AGENT; ANTIOXIDANT; ARIMOCLOMOL; BCL2 RELATED PROTEIN A1; BETA INTERFERON; BIOLOGICAL MARKER; CALCIUM ANTAGONIST; CEFTRIAXONE; CLOZAPINE; CREATINE; DIMETHADIONE; GABAPENTIN; HEAT SHOCK PROTEIN 70; IMMUNOMODULATING AGENT; LAMOTRIGINE; MEMANTINE; MINOCYCLINE; NEUROTOXIN; NEUROTROPHIC FACTOR; NEUROTROPHIN RECEPTOR P75; NICOTINAMIDE ADENINE DINUCLEOTIDE ADENOSINE DIPHOSPHATE RIBOSYLTRANSFERASE INHIBITOR; PENICILLAMINE; PLACEBO; PROTEIN P35; PROTEIN P53; RILUZOLE; TALAMPANEL; TAMOXIFEN; TOPIRAMATE; UNINDEXED DRUG;

EID: 55849140172     PISSN: 02134853     EISSN: 15781968     Source Type: Journal    
DOI: None     Document Type: Review
Times cited : (8)

References (170)
  • 1
    • 36148960127 scopus 로고    scopus 로고
    • Western ALS Study Group. Efficacy of minocycline in patients with amyotrophic lateral sclerosis: A phase III randomized trial
    • Gordon PH, Moore DH, Miller RG, Florence JM, Verheijde JL, Doorish C, et al.; Western ALS Study Group. Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomized trial. Lancet Neurol 2007;6:1045-53.
    • (2007) Lancet Neurol , vol.6 , pp. 1045-1053
    • Gordon, P.H.1    Moore, D.H.2    Miller, R.G.3    Florence, J.M.4    Verheijde, J.L.5    Doorish, C.6
  • 2
    • 33947323759 scopus 로고    scopus 로고
    • Benatar M. Lost in translation: treatment trials in the SOD 1 mouse and in human ALS. Neurobiol Dis 2007;26:1-13.
    • Benatar M. Lost in translation: treatment trials in the SOD 1 mouse and in human ALS. Neurobiol Dis 2007;26:1-13.
  • 3
    • 0024271676 scopus 로고
    • Clinical pharmacokinetics of doxycycline and minocycline
    • Saivin S, Hovin G. Clinical pharmacokinetics of doxycycline and minocycline. Clin Pharmacokinet 1988;15:355-66.
    • (1988) Clin Pharmacokinet , vol.15 , pp. 355-366
    • Saivin, S.1    Hovin, G.2
  • 5
    • 0033539522 scopus 로고    scopus 로고
    • A tetracycline derivative, minoeycline, reduces inflammation and protects against focal cerebral ischemia with a wide therapeutic window
    • Yrjanheikki J, Tikka T, Keinanen R, Goldsteins G, Chan PH, Koistinaho J. A tetracycline derivative, minoeycline, reduces inflammation and protects against focal cerebral ischemia with a wide therapeutic window. Proc Natl Acad Sci USA 1999;96:13496-500.
    • (1999) Proc Natl Acad Sci USA , vol.96 , pp. 13496-13500
    • Yrjanheikki, J.1    Tikka, T.2    Keinanen, R.3    Goldsteins, G.4    Chan, P.H.5    Koistinaho, J.6
  • 7
    • 33646598662 scopus 로고    scopus 로고
    • Minocycline neuroprotects, reduces microgliosis, and inhibits caspase protease expression early after spinal cord injury
    • Festoff BW, Ameenuddin S, Arnold PM, Wong A, Santacruz KS, Citron BA. Minocycline neuroprotects, reduces microgliosis, and inhibits caspase protease expression early after spinal cord injury. J Neurochem 2006;97:1314-26.
    • (2006) J Neurochem , vol.97 , pp. 1314-1326
    • Festoff, B.W.1    Ameenuddin, S.2    Arnold, P.M.3    Wong, A.4    Santacruz, K.S.5    Citron, B.A.6
  • 8
    • 0037007645 scopus 로고    scopus 로고
    • Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice
    • Zhu S, Stavrovskaya IG, Drozda M, Kim BY, Ona V, Li M, et al. Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice. Nature 2002;417:74-8.
    • (2002) Nature , vol.417 , pp. 74-78
    • Zhu, S.1    Stavrovskaya, I.G.2    Drozda, M.3    Kim, B.Y.4    Ona, V.5    Li, M.6
  • 9
    • 0036406903 scopus 로고    scopus 로고
    • Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis
    • Kriz J, Nguyen M, Julien J. Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 2002;10:268-78.
    • (2002) Neurobiol Dis , vol.10 , pp. 268-278
    • Kriz, J.1    Nguyen, M.2    Julien, J.3
  • 10
    • 0037067009 scopus 로고    scopus 로고
    • Minocycline delays disease onset and mortality in a transgenic model of ALS
    • Van Den Bosch L, Tilkin P, Lemmens G, Robberecht W. Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport 2002;13:1067-70.
    • (2002) Neuroreport , vol.13 , pp. 1067-1070
    • Van Den Bosch, L.1    Tilkin, P.2    Lemmens, G.3    Robberecht, W.4
  • 11
    • 0036209082 scopus 로고    scopus 로고
    • Minocycline prevents neurotoxicity induced by cerebrospinal fluid patients with motor neurone disease
    • Tikka TM, Vartianen NE, Goldsteins G, Oja SO, Andersen PM, Marklund SL, et al. Minocycline prevents neurotoxicity induced by cerebrospinal fluid patients with motor neurone disease. Brain 2002;722-31.
    • (2002) Brain , pp. 722-731
    • Tikka, T.M.1    Vartianen, N.E.2    Goldsteins, G.3    Oja, S.O.4    Andersen, P.M.5    Marklund, S.L.6
  • 12
    • 0036210054 scopus 로고    scopus 로고
    • Toxicity of CSF in motor neurone disease: A potential route to neuroprotection
    • Shaw PJ. Toxicity of CSF in motor neurone disease: a potential route to neuroprotection. Brain 2002;25:693-4.
    • (2002) Brain , vol.25 , pp. 693-694
    • Shaw, P.J.1
  • 13
    • 0033912716 scopus 로고    scopus 로고
    • Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
    • Chen M, Ona VO, Li M, Ferrante RJ, Fink KB, Zhu S, et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med 2000;6:797-801.
    • (2000) Nat Med , vol.6 , pp. 797-801
    • Chen, M.1    Ona, V.O.2    Li, M.3    Ferrante, R.J.4    Fink, K.B.5    Zhu, S.6
  • 14
    • 0041335559 scopus 로고    scopus 로고
    • Minocycline inhibits caspase-independent and dependent mitochondrial cell death pathways in models of Huntington's disease
    • Wang X, Zhu S, Drozda M, Zhang W, Stavrovskaya IG, Cattaneo E, et al. Minocycline inhibits caspase-independent and dependent mitochondrial cell death pathways in models of Huntington's disease. Proc Natl Acad Sci USA 2003;100:10483-7.
    • (2003) Proc Natl Acad Sci USA , vol.100 , pp. 10483-10487
    • Wang, X.1    Zhu, S.2    Drozda, M.3    Zhang, W.4    Stavrovskaya, I.G.5    Cattaneo, E.6
  • 15
    • 0036260089 scopus 로고    scopus 로고
    • Targeting leukocyte MMP and transmigration: Minocycline as a potential therapy for multiple sclerosis
    • Brundula V, Rewcastle NB, Metz LM, Bernard CC, Yong VW. Targeting leukocyte MMP and transmigration: minocycline as a potential therapy for multiple sclerosis. Brain 2002;125:1297-308.
    • (2002) Brain , vol.125 , pp. 1297-1308
    • Brundula, V.1    Rewcastle, N.B.2    Metz, L.M.3    Bernard, C.C.4    Yong, V.W.5
  • 17
    • 0036830527 scopus 로고    scopus 로고
    • Effect of minocycline in experimental autoimmune encephalomyelitis
    • Nessler S, Dodel R, Bittner A, Reuss S, Du Y, Hemmer B, et al. Effect of minocycline in experimental autoimmune encephalomyelitis. Ann Neurol 2002;52:689-90.
    • (2002) Ann Neurol , vol.52 , pp. 689-690
    • Nessler, S.1    Dodel, R.2    Bittner, A.3    Reuss, S.4    Du, Y.5    Hemmer, B.6
  • 18
    • 0037481943 scopus 로고    scopus 로고
    • Neuroprotection by minocycline facilitates significant recovery from spinal cord injury
    • Wells J, Hurlbert J, Fehlings M, Yong VW. Neuroprotection by minocycline facilitates significant recovery from spinal cord injury. Brain 2003;126:1628-37.
    • (2003) Brain , vol.126 , pp. 1628-1637
    • Wells, J.1    Hurlbert, J.2    Fehlings, M.3    Yong, V.W.4
  • 19
    • 0035807904 scopus 로고    scopus 로고
    • Minocycline prevents nigrostriatal dopaminergic neurodegeneration in the MPTP model of Parkinson's disease
    • Du Y, Ma Z, Lin S, Dodel RC, Gao F, Bales KR, et al. Minocycline prevents nigrostriatal dopaminergic neurodegeneration in the MPTP model of Parkinson's disease. Proc Natl Acad Sci USA 2001;98:14669-74.
    • (2001) Proc Natl Acad Sci USA , vol.98 , pp. 14669-14674
    • Du, Y.1    Ma, Z.2    Lin, S.3    Dodel, R.C.4    Gao, F.5    Bales, K.R.6
  • 20
    • 9144267928 scopus 로고    scopus 로고
    • Minocycline prevents cholinergic loss in a mouse model of Down's syndrome
    • Hunter CL, Bachman D, Granholm AC. Minocycline prevents cholinergic loss in a mouse model of Down's syndrome. Ann Neurol 2004;56:675-88.
    • (2004) Ann Neurol , vol.56 , pp. 675-688
    • Hunter, C.L.1    Bachman, D.2    Granholm, A.C.3
  • 21
    • 3543092682 scopus 로고    scopus 로고
    • Minocycline safety and tolerability in Huntington disease
    • Huntington Study Group
    • Huntington Study Group. Minocycline safety and tolerability in Huntington disease. Neurology 2004;63:547-9.
    • (2004) Neurology , vol.63 , pp. 547-549
  • 22
    • 17544370486 scopus 로고    scopus 로고
    • Neuroprotective and anti-human immunodeficiency virus activity of minocycline
    • Zink MC, Uhrlaub J, DeWitt J, Voelker T, Bullock B, Mankowski J, et al. Neuroprotective and anti-human immunodeficiency virus activity of minocycline. JAMA 2005;293:2003-11.
    • (2005) JAMA , vol.293 , pp. 2003-2011
    • Zink, M.C.1    Uhrlaub, J.2    DeWitt, J.3    Voelker, T.4    Bullock, B.5    Mankowski, J.6
  • 23
    • 0037310563 scopus 로고    scopus 로고
    • Additive neuroprotective effects of minocycline with creatine in a mouse model of ALS
    • Zhang W, Narayanan M, Friedlander RM. Additive neuroprotective effects of minocycline with creatine in a mouse model of ALS. Ann Neurol 2003;53:267-70.
    • (2003) Ann Neurol , vol.53 , pp. 267-270
    • Zhang, W.1    Narayanan, M.2    Friedlander, R.M.3
  • 24
    • 0037381851 scopus 로고    scopus 로고
    • Efficient three-drug cocktail for disease induced by mutant superoxide dismutase
    • Kriz J, Gowing G, Julien JP. Efficient three-drug cocktail for disease induced by mutant superoxide dismutase. Ann Neurol 2003;53:429-36.
    • (2003) Ann Neurol , vol.53 , pp. 429-436
    • Kriz, J.1    Gowing, G.2    Julien, J.P.3
  • 25
    • 34548637868 scopus 로고    scopus 로고
    • Minocycline and riluzole brain disposition: Interactions with p-glycoprotein at the blood-brain barrier
    • Milane A, Fernández C, Vautier S, Bensimon G, Meininger V, Farinotti R. Minocycline and riluzole brain disposition: interactions with p-glycoprotein at the blood-brain barrier. J Neurochem 2007;103:164-73.
    • (2007) J Neurochem , vol.103 , pp. 164-173
    • Milane, A.1    Fernández, C.2    Vautier, S.3    Bensimon, G.4    Meininger, V.5    Farinotti, R.6
  • 26
    • 33646243562 scopus 로고    scopus 로고
    • Minocycline affects microglia activation, Abeta deposition, and behavior in APP-tg mice
    • Seabrook TJ, Jiang L, Maier M, Lemere CA. Minocycline affects microglia activation, Abeta deposition, and behavior in APP-tg mice. Glia 2006;53:776-82.
    • (2006) Glia , vol.53 , pp. 776-782
    • Seabrook, T.J.1    Jiang, L.2    Maier, M.3    Lemere, C.A.4
  • 27
    • 36448996084 scopus 로고    scopus 로고
    • ALS trial raises questions about promising drug
    • Couzin J. ALS trial raises questions about promising drug. Science 2007;318:1227.
    • (2007) Science , vol.318 , pp. 1227
    • Couzin, J.1
  • 28
    • 2442715084 scopus 로고    scopus 로고
    • Placebo-controlled phase I/II studies of minocycline in amyotrophic lateral sclerosis
    • Gordon PH, Moore DH, Gelinas DF, Qualls C, Meister ME, Werner J, et al. Placebo-controlled phase I/II studies of minocycline in amyotrophic lateral sclerosis. Neurology 2004;62:1845-7.
    • (2004) Neurology , vol.62 , pp. 1845-1847
    • Gordon, P.H.1    Moore, D.H.2    Gelinas, D.F.3    Qualls, C.4    Meister, M.E.5    Werner, J.6
  • 30
    • 22144496427 scopus 로고    scopus 로고
    • Effective combination of minocycline and interferon-beta in a model of multiple sclerosis
    • Giuliani F, Fu SA, Metz LM, Yong VW. Effective combination of minocycline and interferon-beta in a model of multiple sclerosis. J Neuroimmunol 2005;165:83-91.
    • (2005) J Neuroimmunol , vol.165 , pp. 83-91
    • Giuliani, F.1    Fu, S.A.2    Metz, L.M.3    Yong, V.W.4
  • 31
    • 13944257807 scopus 로고    scopus 로고
    • Minocycline in neurological diseases
    • Bandini F. Minocycline in neurological diseases. Lancet Neurol 2005,4:138-9.
    • (2005) Lancet Neurol , vol.4 , pp. 138-139
    • Bandini, F.1
  • 33
    • 33847696170 scopus 로고    scopus 로고
    • Revisiting safety of minocycline neuroprotection in Huntington's disease
    • Reynolds N. Revisiting safety of minocycline neuroprotection in Huntington's disease. Mov Disord 2007;22:292.
    • (2007) Mov Disord , vol.22 , pp. 292
    • Reynolds, N.1
  • 34
    • 34848857092 scopus 로고    scopus 로고
    • Minocylcine treatment in acute stroke. An open-label, evaluator-blinder study
    • Lampl Y, Boaz M, Gilad R, Lorberboym M, Dabby R, Rapoport A, et al. Minocylcine treatment in acute stroke. An open-label, evaluator-blinder study. Neurology 2007;69:1404-10.
    • (2007) Neurology , vol.69 , pp. 1404-1410
    • Lampl, Y.1    Boaz, M.2    Gilad, R.3    Lorberboym, M.4    Dabby, R.5    Rapoport, A.6
  • 35
    • 3042755065 scopus 로고    scopus 로고
    • Deleterious effects of minocycline in animal models of Parkinson's disease and Huntington's disease
    • Diguet E, Fernagut PO, Wei X, Du Y, Rouland R, Gross C, et al. Deleterious effects of minocycline in animal models of Parkinson's disease and Huntington's disease. Eur J Neurosci 2004;19:3266-76.
    • (2004) Eur J Neurosci , vol.19 , pp. 3266-3276
    • Diguet, E.1    Fernagut, P.O.2    Wei, X.3    Du, Y.4    Rouland, R.5    Gross, C.6
  • 36
    • 3543073536 scopus 로고    scopus 로고
    • Minocycline worsens hypoxic-ischemic brain injury in a neonatal mouse model
    • Tsuji M, Wilson MA, Lange MS, Johnston MV. Minocycline worsens hypoxic-ischemic brain injury in a neonatal mouse model. Exp Neurol 2004;189:58-65.
    • (2004) Exp Neurol , vol.189 , pp. 58-65
    • Tsuji, M.1    Wilson, M.A.2    Lange, M.S.3    Johnston, M.V.4
  • 38
    • 0026472983 scopus 로고
    • What do we really know about amyotrophic lateral sclerosis?
    • Swash M, Schwartz MS. What do we really know about amyotrophic lateral sclerosis? J Neurol Sci 1992;113:4-16.
    • (1992) J Neurol Sci , vol.113 , pp. 4-16
    • Swash, M.1    Schwartz, M.S.2
  • 39
    • 0028998398 scopus 로고
    • Amyotrophic lateral sclerosis is a multifactorial disease
    • Eisen A. Amyotrophic lateral sclerosis is a multifactorial disease. Muscle Nerve 1995;18:741-5.
    • (1995) Muscle Nerve , vol.18 , pp. 741-745
    • Eisen, A.1
  • 40
    • 0029661207 scopus 로고    scopus 로고
    • A controlled trial of amino acid therapy in amyotrophic lateral sclerosis: I. Clinical, functional, and maximum isometric torque data
    • Tandan R, Bromberg MB, Forshew D, Fries TJ, Badger GJ, Carpenter J, et al. A controlled trial of amino acid therapy in amyotrophic lateral sclerosis: I. Clinical, functional, and maximum isometric torque data. Neurology 1996;47:1220-6.
    • (1996) Neurology , vol.47 , pp. 1220-1226
    • Tandan, R.1    Bromberg, M.B.2    Forshew, D.3    Fries, T.J.4    Badger, G.J.5    Carpenter, J.6
  • 41
    • 3142514196 scopus 로고    scopus 로고
    • Oxidative stress in neurodegeneration: Cause or consequence?
    • Andersen JK. Oxidative stress in neurodegeneration: cause or consequence? Nat Med 2004;10(Suppl.):S18-25.
    • (2004) Nat Med , vol.10 , Issue.SUPPL.
    • Andersen, J.K.1
  • 44
    • 0029969678 scopus 로고    scopus 로고
    • Mechanisms of selective motor neuron death in ALS: Insights from transgenic mouse models of motor neuron disease
    • Bruijn LI, Cleveland DW. Mechanisms of selective motor neuron death in ALS: insights from transgenic mouse models of motor neuron disease. Neuropathol Appl Neurobiol 1996;22:373-87.
    • (1996) Neuropathol Appl Neurobiol , vol.22 , pp. 373-387
    • Bruijn, L.I.1    Cleveland, D.W.2
  • 45
    • 3943102116 scopus 로고    scopus 로고
    • Unravelling the mechanisms involved in motor neuron degeneration in ALS
    • Bruijn LI, Miller TM, Cleveland DW. Unravelling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci 2004;27:723-49.
    • (2004) Annu Rev Neurosci , vol.27 , pp. 723-749
    • Bruijn, L.I.1    Miller, T.M.2    Cleveland, D.W.3
  • 46
    • 0035516124 scopus 로고    scopus 로고
    • From Charcot to Lou Gehrig: Deciphering selective motor neuron death in ALS
    • Cleveland DW, Rothstein JD. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci 2001;2:806-18.
    • (2001) Nat Rev Neurosci , vol.2 , pp. 806-818
    • Cleveland, D.W.1    Rothstein, J.D.2
  • 47
    • 12344286247 scopus 로고    scopus 로고
    • The pro and the active form of matrix metalloproteinase-9 is increased in serum of patients with amyotrophic lateral sclerosis
    • Demestre M, Parkin-Smith G, Petzold A, Pullen AH. The pro and the active form of matrix metalloproteinase-9 is increased in serum of patients with amyotrophic lateral sclerosis. J Neuroimmunol 2005;159:146-54.
    • (2005) J Neuroimmunol , vol.159 , pp. 146-154
    • Demestre, M.1    Parkin-Smith, G.2    Petzold, A.3    Pullen, A.H.4
  • 48
    • 0027426169 scopus 로고
    • Amyotrophic lateral sclerosis and structural defects in Cu, Zn superoxide dismutase
    • Deng HX, Hentati A, Tainer JA, Iqbal Z, Cayabyab A, Hung WY, et al. Amyotrophic lateral sclerosis and structural defects in Cu, Zn superoxide dismutase. Science 1993;261:1047-51.
    • (1993) Science , vol.261 , pp. 1047-1051
    • Deng, H.X.1    Hentati, A.2    Tainer, J.A.3    Iqbal, Z.4    Cayabyab, A.5    Hung, W.Y.6
  • 49
    • 34247473080 scopus 로고    scopus 로고
    • Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model
    • Di Giorgio FP, Carrasco MA, Siao MC, Maniatis T, Eggan K. Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model. Nat Neurosc 2007;10:608-14.
    • (2007) Nat Neurosc , vol.10 , pp. 608-614
    • Di Giorgio, F.P.1    Carrasco, M.A.2    Siao, M.C.3    Maniatis, T.4    Eggan, K.5
  • 50
    • 33746303958 scopus 로고    scopus 로고
    • Mutational analysis of the Cu/Zn Superoxide dismutase gene in a Catalan ALS population: Should all sporadic ALS cases also be screened for SOD1?
    • Gámez J, Corbera-Bellalta M, Nogales G, Raguer N, Garcia-Arumí E, Badía-Canto M, et al. Mutational analysis of the Cu/Zn Superoxide dismutase gene in a Catalan ALS population: Should all sporadic ALS cases also be screened for SOD1? J Neurol Sci 2006;247:21-8.
    • (2006) J Neurol Sci , vol.247 , pp. 21-28
    • Gámez, J.1    Corbera-Bellalta, M.2    Nogales, G.3    Raguer, N.4    Garcia-Arumí, E.5    Badía-Canto, M.6
  • 51
    • 0037069237 scopus 로고    scopus 로고
    • Survival and respiratory decline are not related to homozygous SMN2 deletions in ALS patients
    • Gámez J, Barceló MJ, Muñoz X, Carmona F, Cuscó I, Baiget M, et al. Survival and respiratory decline are not related to homozygous SMN2 deletions in ALS patients. Neurology 2002;59:1456-60.
    • (2002) Neurology , vol.59 , pp. 1456-1460
    • Gámez, J.1    Barceló, M.J.2    Muñoz, X.3    Carmona, F.4    Cuscó, I.5    Baiget, M.6
  • 53
    • 0034647003 scopus 로고    scopus 로고
    • Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model
    • Li M, Ona VO, Guégan C, Chen M, Jackson-Lewis V, Andrews LJ, et al. Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model. Science 2000;288:335-9.
    • (2000) Science , vol.288 , pp. 335-339
    • Li, M.1    Ona, V.O.2    Guégan, C.3    Chen, M.4    Jackson-Lewis, V.5    Andrews, L.J.6
  • 54
    • 41449113885 scopus 로고    scopus 로고
    • Altered macroautophagy in the spinal cord of SOD1 mutant mice
    • Li L, Zhang X, Le W. Altered macroautophagy in the spinal cord of SOD1 mutant mice. Autophagy 2008;4:290-3.
    • (2008) Autophagy , vol.4 , pp. 290-293
    • Li, L.1    Zhang, X.2    Le, W.3
  • 55
    • 0032942541 scopus 로고    scopus 로고
    • Increased hydroxyl radical production and apoptosis in PC12 neuron cells expressing the gain-of-function mutant G93A SOD1 gene
    • Liu R, Narla RK, Kurinov I, Li B, Uckun FM. Increased hydroxyl radical production and apoptosis in PC12 neuron cells expressing the gain-of-function mutant G93A SOD1 gene. Radiat Res 1999;151:133-41.
    • (1999) Radiat Res , vol.151 , pp. 133-141
    • Liu, R.1    Narla, R.K.2    Kurinov, I.3    Li, B.4    Uckun, F.M.5
  • 56
    • 17344366821 scopus 로고    scopus 로고
    • Astroglial alterations in amyotrophic lateral sclerosis (ALS) model of slow glutamate excitotoxicity in vitro
    • Matyja E, Taraszewska A, Naganska E, Rafalowska J, Gebarowska J. Astroglial alterations in amyotrophic lateral sclerosis (ALS) model of slow glutamate excitotoxicity in vitro. Folia Neuropathol 2005;43:7-13.
    • (2005) Folia Neuropathol , vol.43 , pp. 7-13
    • Matyja, E.1    Taraszewska, A.2    Naganska, E.3    Rafalowska, J.4    Gebarowska, J.5
  • 57
    • 0037444453 scopus 로고    scopus 로고
    • Cell cycle regulators in the neuronal death pathway of amyotrophic lateral sclerosis caused by mutant superoxide dismutase 1
    • Nguyen MD, Boudreau M, Kriz J, Couillard-Després S, Kaplan DR, Julien JP. Cell cycle regulators in the neuronal death pathway of amyotrophic lateral sclerosis caused by mutant superoxide dismutase 1. J Neurosci 2003;23:2131-40.
    • (2003) J Neurosci , vol.23 , pp. 2131-2140
    • Nguyen, M.D.1    Boudreau, M.2    Kriz, J.3    Couillard-Després, S.4    Kaplan, D.R.5    Julien, J.P.6
  • 58
    • 0034978562 scopus 로고    scopus 로고
    • Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration
    • Oosthuyse B, Moons L, Storkebaum E, Beck H, Nuyens D, Brusselmans K, et al. Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration. Nat Genet 2001;28:131-8.
    • (2001) Nat Genet , vol.28 , pp. 131-138
    • Oosthuyse, B.1    Moons, L.2    Storkebaum, E.3    Beck, H.4    Nuyens, D.5    Brusselmans, K.6
  • 59
    • 0027401203 scopus 로고
    • Mutations in Cu/Zn superoxide dismutase are associated with familial amyotrophic lateral sclerosis
    • Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, et al. Mutations in Cu/Zn superoxide dismutase are associated with familial amyotrophic lateral sclerosis. Nature 1993;362:59-62.
    • (1993) Nature , vol.362 , pp. 59-62
    • Rosen, D.R.1    Siddique, T.2    Patterson, D.3    Figlewicz, D.A.4    Sapp, P.5    Hentati, A.6
  • 60
    • 0035978743 scopus 로고    scopus 로고
    • Amyotrophic lateral sclerosis
    • Rowland LP, Shneider NA. Amyotrophic lateral sclerosis. N Engl J Med 2001;344:1688-700.
    • (2001) N Engl J Med , vol.344 , pp. 1688-1700
    • Rowland, L.P.1    Shneider, N.A.2
  • 61
    • 41149180753 scopus 로고    scopus 로고
    • TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
    • Sreedharan J, Blair IP, Tripathi VB, Hu X, Vance C, Rogelj B, et al. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 2008;319:1668-72.
    • (2008) Science , vol.319 , pp. 1668-1672
    • Sreedharan, J.1    Blair, I.P.2    Tripathi, V.B.3    Hu, X.4    Vance, C.5    Rogelj, B.6
  • 62
    • 34547562663 scopus 로고    scopus 로고
    • EALSC Working Group. Good practice in the management of amyotrophic lateral sclerosis: Clinical guidelines. An evidence-based review with good practice points. EALSC Working Group
    • Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN, et al.; EALSC Working Group. Good practice in the management of amyotrophic lateral sclerosis: clinical guidelines. An evidence-based review with good practice points. EALSC Working Group. Amyotroph Lateral Scler 2007;8:195-213.
    • (2007) Amyotroph Lateral Scler , vol.8 , pp. 195-213
    • Andersen, P.M.1    Borasio, G.D.2    Dengler, R.3    Hardiman, O.4    Kollewe, K.5    Leigh, P.N.6
  • 63
    • 0035023789 scopus 로고    scopus 로고
    • Motor neuron cell death and neurotrophic factors: Basic models for development of new therapeutic strategies in ALS
    • Beck M, Karch C, Wiese S, Sendtne M. Motor neuron cell death and neurotrophic factors: basic models for development of new therapeutic strategies in ALS. Amyotrophic Lateral Scler Other Motor Neuron Disord 2001;2(Suppl. 1):55-68.
    • (2001) Amyotrophic Lateral Scler Other Motor Neuron Disord , vol.2 , Issue.SUPPL. 1 , pp. 55-68
    • Beck, M.1    Karch, C.2    Wiese, S.3    Sendtne, M.4
  • 64
    • 0021253595 scopus 로고
    • A double-blind controlled trial of bovine brain gangliosides in amyotrophic lateral sclerosis
    • Bradley WG, Hedlund W, Cooper C, Desousa GJ, Gabbai A, Mora JS, et al. A double-blind controlled trial of bovine brain gangliosides in amyotrophic lateral sclerosis. Neurology 1984;34:1079-082.
    • (1984) Neurology , vol.34 , pp. 1079-1082
    • Bradley, W.G.1    Hedlund, W.2    Cooper, C.3    Desousa, G.J.4    Gabbai, A.5    Mora, J.S.6
  • 66
    • 0030898561 scopus 로고    scopus 로고
    • Clinical evaluation of ALS drugs
    • Brooks BJ. Clinical evaluation of ALS drugs. Neurology 1997; 48(Suppl. 4):S23-7.
    • (1997) Neurology , vol.48 , Issue.SUPPL. 4
    • Brooks, B.J.1
  • 67
    • 0032227330 scopus 로고    scopus 로고
    • Reduced glutathione in amyotrophic lateral sclerosis: An open, cross-over, randomized trial
    • Chiò A, Cucatto A, Terreni A, Schiffer D. Reduced glutathione in amyotrophic lateral sclerosis: an open, cross-over, randomized trial. Ital J Neurol Sci 1998;19:363-6.
    • (1998) Ital J Neurol Sci , vol.19 , pp. 363-366
    • Chiò, A.1    Cucatto, A.2    Terreni, A.3    Schiffer, D.4
  • 68
    • 0035007860 scopus 로고    scopus 로고
    • A double-blind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study Group
    • Desnuelle C, Dib M, Garrel C, Favier A. A double-blind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study Group. Amyotroph Lateral Scler Other Motor Neuron Disord 2001;2:9-18.
    • (2001) Amyotroph Lateral Scler Other Motor Neuron Disord , vol.2 , pp. 9-18
    • Desnuelle, C.1    Dib, M.2    Garrel, C.3    Favier, A.4
  • 69
    • 0028121750 scopus 로고
    • Trial of immunosuppression in amyotrophic lateral sclerosis using total lymphoid irradiation
    • Drachmen DB, Chaudhry V, Cornblath D, Kuncl RW, Pestronk A, Clawson L, et al. Trial of immunosuppression in amyotrophic lateral sclerosis using total lymphoid irradiation. Ann Neurol 1994;35:142-50.
    • (1994) Ann Neurol , vol.35 , pp. 142-150
    • Drachmen, D.B.1    Chaudhry, V.2    Cornblath, D.3    Kuncl, R.W.4    Pestronk, A.5    Clawson, L.6
  • 70
    • 0028121750 scopus 로고
    • Trial of immunosuppression in amyotrophic lateral sclerosis using total lymphoid irradiation
    • Drachman DB, Chaudhry V, Cornblath D, Kuncl RW, Pestronk A, Clawson L, et al. Trial of immunosuppression in amyotrophic lateral sclerosis using total lymphoid irradiation. Ann Neurol 1994;35:142-50.
    • (1994) Ann Neurol , vol.35 , pp. 142-150
    • Drachman, D.B.1    Chaudhry, V.2    Cornblath, D.3    Kuncl, R.W.4    Pestronk, A.5    Clawson, L.6
  • 71
    • 0027759524 scopus 로고
    • Anti-glutamate therapy in amyotrophic lateral sclerosis: A trial using lamotrigine
    • Eisen A, Stewart H, Schulzer M, Cameron D. Anti-glutamate therapy in amyotrophic lateral sclerosis: a trial using lamotrigine. Can J Neurol Sci 1993;20:297-301.
    • (1993) Can J Neurol Sci , vol.20 , pp. 297-301
    • Eisen, A.1    Stewart, H.2    Schulzer, M.3    Cameron, D.4
  • 72
    • 0030833791 scopus 로고    scopus 로고
    • Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis
    • Gourie-Devi M, Nalini A, Subbakrishna DK. Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis. J Neurol Sci 1997;150:167-72.
    • (1997) J Neurol Sci , vol.150 , pp. 167-172
    • Gourie-Devi, M.1    Nalini, A.2    Subbakrishna, D.K.3
  • 73
    • 17844387640 scopus 로고    scopus 로고
    • German vitamin E/ALS Study Group. High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: Results of a placebo-controlled double-blind study
    • Graf M, Ecker D, Horowski R, Kramer B, Riederer P, Gerlach M, et al.; German vitamin E/ALS Study Group. High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: results of a placebo-controlled double-blind study. J Neural Transm 2005;112:649-60.
    • (2005) J Neural Transm , vol.112 , pp. 649-660
    • Graf, M.1    Ecker, D.2    Horowski, R.3    Kramer, B.4    Riederer, P.5    Gerlach, M.6
  • 74
    • 0021241929 scopus 로고
    • Ganglioside therapy for amyotrophic lateral sclerosis: A double-blind controlled trial
    • Harrington H, Hallett M, Tyler HR. Ganglioside therapy for amyotrophic lateral sclerosis: a double-blind controlled trial. Neurology 1984;34:1083-5.
    • (1984) Neurology , vol.34 , pp. 1083-1085
    • Harrington, H.1    Hallett, M.2    Tyler, H.R.3
  • 75
    • 0034326241 scopus 로고    scopus 로고
    • Riluzole prolongs survival and delays muscle strength deterioration in mice with progressive motor neuronopathy (pmn)
    • Kennel P, Revah F, Bohme GA, Bejuit R, Gallix P, Stutzmann JM, et al. Riluzole prolongs survival and delays muscle strength deterioration in mice with progressive motor neuronopathy (pmn). J Neurol Sci 2000;180:55-61.
    • (2000) J Neurol Sci , vol.180 , pp. 55-61
    • Kennel, P.1    Revah, F.2    Bohme, G.A.3    Bejuit, R.4    Gallix, P.5    Stutzmann, J.M.6
  • 76
    • 0028097839 scopus 로고
    • A controlled trial of riluzole in amyotrophic lateral sclerosis
    • and The ALS/Riluzole Study Group
    • Bensimon G, Lacomblez L, Meininger V, and The ALS/Riluzole Study Group. A controlled trial of riluzole in amyotrophic lateral sclerosis. N Engl J Med 1994;330:585-91.
    • (1994) N Engl J Med , vol.330 , pp. 585-591
    • Bensimon, G.1    Lacomblez, L.2    Meininger, V.3
  • 78
    • 0029977337 scopus 로고    scopus 로고
    • Dose-ranging study of riluzole in amyotrophic lateral sclerosis
    • for the Amyotrophic Lateral Sclerosis/Riluzole Study Group II
    • Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V, for the Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Lancet 1996;347:1425-31.
    • (1996) Lancet , vol.347 , pp. 1425-1431
    • Lacomblez, L.1    Bensimon, G.2    Leigh, P.N.3    Guillet, P.4    Meininger, V.5
  • 79
    • 6844266272 scopus 로고    scopus 로고
    • Effect of recombinant human insulin-like growth factor-I on progression of ALS. A placebo-controlled study. The North America ALS/IGF-I Study Group
    • Lai EC, Felice KJ, Festoff BW, Gawel MJ, Gelinas DF, Kratz R, et al. Effect of recombinant human insulin-like growth factor-I on progression of ALS. A placebo-controlled study. The North America ALS/IGF-I Study Group. Neurology 1997;49:1621-30.
    • (1997) Neurology , vol.49 , pp. 1621-1630
    • Lai, E.C.1    Felice, K.J.2    Festoff, B.W.3    Gawel, M.J.4    Gelinas, D.F.5    Kratz, R.6
  • 80
    • 34249802918 scopus 로고    scopus 로고
    • A controlled study of reverse transcriptase in serum and CSF of HIV-negative patients with ALS
    • MacGowan DJ, Scelsa SN, Imperato TE, Liu KN, Baron P, Polsky B. A controlled study of reverse transcriptase in serum and CSF of HIV-negative patients with ALS. Neurology 2007;68:1944-6.
    • (2007) Neurology , vol.68 , pp. 1944-1946
    • MacGowan, D.J.1    Scelsa, S.N.2    Imperato, T.E.3    Liu, K.N.4    Baron, P.5    Polsky, B.6
  • 81
    • 38549107484 scopus 로고    scopus 로고
    • Quantification of reverse transcriptase in ALS and elimination of a novel retroviral candidate
    • McCormick AL, Brown Jr. RH, Cudkowicz ME, Al-Chalabi A, Garson JA. Quantification of reverse transcriptase in ALS and elimination of a novel retroviral candidate. Neurology 2008;70:278-83.
    • (2008) Neurology , vol.70 , pp. 278-283
    • McCormick, A.L.1    Brown Jr., R.H.2    Cudkowicz, M.E.3    Al-Chalabi, A.4    Garson, J.A.5
  • 82
    • 20144385858 scopus 로고    scopus 로고
    • Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis mice
    • Ryu H, Smith K, Camelo SI, Carreras I, Lee J, Iglesias AH, et al. Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis mice. J Neurochem 2005;93:1087-98.
    • (2005) J Neurochem , vol.93 , pp. 1087-1098
    • Ryu, H.1    Smith, K.2    Camelo, S.I.3    Carreras, I.4    Lee, J.5    Iglesias, A.H.6
  • 83
    • 33746849034 scopus 로고    scopus 로고
    • Neuroprotective agents for clinical trials in ALS. A systematic assessment
    • Traynor BJ, Bruijn L, Conwit R, Beal F, O'Neill G, Fagan SC, et al. Neuroprotective agents for clinical trials in ALS. A systematic assessment. Neurology 2006;67:20-7.
    • (2006) Neurology , vol.67 , pp. 20-27
    • Traynor, B.J.1    Bruijn, L.2    Conwit, R.3    Beal, F.4    O'Neill, G.5    Fagan, S.C.6
  • 84
    • 20244381261 scopus 로고    scopus 로고
    • Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model
    • Ralph GS, Radcliffe PA, Day DM, Carthy JM, Leroux MA, Lee DC, et al. Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model. Nat Med 2005;11:429-33.
    • (2005) Nat Med , vol.11 , pp. 429-433
    • Ralph, G.S.1    Radcliffe, P.A.2    Day, D.M.3    Carthy, J.M.4    Leroux, M.A.5    Lee, D.C.6
  • 85
    • 33747201641 scopus 로고    scopus 로고
    • Allele-specific RNAi selectively silences mutant SOD1 and achieves significant therapeutic benefit in vivo
    • Xia X, Zhou H, Huang Y, Xu Z. Allele-specific RNAi selectively silences mutant SOD1 and achieves significant therapeutic benefit in vivo. Neurobiol Dis 2006;23:578-86.
    • (2006) Neurobiol Dis , vol.23 , pp. 578-586
    • Xia, X.1    Zhou, H.2    Huang, Y.3    Xu, Z.4
  • 87
    • 1542374572 scopus 로고    scopus 로고
    • Mécanismes moléculaires de la sclérose latérale amyotrophique: Apports récents de l'analyse de modèles animaux.
    • Dupuis L, Muller A, Meininger V, Loeffler JP. Mécanismes moléculaires de la sclérose latérale amyotrophique: apports récents de l'analyse de modèles animaux. Rev Neurol (Paris) 2004;160:35-43.
    • (2004) Rev Neurol (Paris) , vol.160 , pp. 35-43
    • Dupuis, L.1    Muller, A.2    Meininger, V.3    Loeffler, J.P.4
  • 88
    • 0036838167 scopus 로고    scopus 로고
    • Increased plasma TGF-1 in patients with amyotrophic lateral sclerosis
    • Houi K, Kobayashi T, Kato S, Mochio S, Inoue K. Increased plasma TGF-1 in patients with amyotrophic lateral sclerosis. Acta Neurol Scand 2002;106:299-301.
    • (2002) Acta Neurol Scand , vol.106 , pp. 299-301
    • Houi, K.1    Kobayashi, T.2    Kato, S.3    Mochio, S.4    Inoue, K.5
  • 89
    • 0042168943 scopus 로고    scopus 로고
    • Prostaglandin E2 is increased in amyotrophic lateral sclerosis patients
    • Ilzecka J. Prostaglandin E2 is increased in amyotrophic lateral sclerosis patients. Acta Neurol Scand 2003;108:125-9.
    • (2003) Acta Neurol Scand , vol.108 , pp. 125-129
    • Ilzecka, J.1
  • 90
    • 33746933794 scopus 로고    scopus 로고
    • Cerebrospinal fluid Flt3 ligand level in patients with amyotrophic lateral sclerosis
    • Ilzecka J. Cerebrospinal fluid Flt3 ligand level in patients with amyotrophic lateral sclerosis. Acta Neurol Scand 2006;114:205-9.
    • (2006) Acta Neurol Scand , vol.114 , pp. 205-209
    • Ilzecka, J.1
  • 91
    • 34247402569 scopus 로고    scopus 로고
    • Decreased cerebrospinal fluid cytochrome c levels in patients with amyotrophic lateral sclerosis
    • Ilzecka J. Decreased cerebrospinal fluid cytochrome c levels in patients with amyotrophic lateral sclerosis. Scand J Clin Lab Invest 2007;67:264-9.
    • (2007) Scand J Clin Lab Invest , vol.67 , pp. 264-269
    • Ilzecka, J.1
  • 92
    • 46949083065 scopus 로고    scopus 로고
    • Decreased serum endoglin level in patients with amyotrophic lateral sclerosis: A preliminary report
    • Ilzecka J. Decreased serum endoglin level in patients with amyotrophic lateral sclerosis: a preliminary report. Scand J Clin Lab Invest 2008;68:1-5.
    • (2008) Scand J Clin Lab Invest , vol.68 , pp. 1-5
    • Ilzecka, J.1
  • 93
    • 41849101398 scopus 로고    scopus 로고
    • Decreased serum-soluble TRAIL levels in patients with amyotrophic lateral sclerosis
    • Ilzecka J. Decreased serum-soluble TRAIL levels in patients with amyotrophic lateral sclerosis. Acta Neurol Scand 2008; 117:343-6.
    • (2008) Acta Neurol Scand , vol.117 , pp. 343-346
    • Ilzecka, J.1
  • 94
    • 37149006825 scopus 로고    scopus 로고
    • Elevation of MCP-1 and MCP-1/VEGF ratio in cerebrospinal fluid of amyotrophic lateral sclerosis patients
    • Nagata T, Nagano I, Shiote M, Narai H, Murakami T, Hayashi T, et al. Elevation of MCP-1 and MCP-1/VEGF ratio in cerebrospinal fluid of amyotrophic lateral sclerosis patients. Neurol Res 2007;29:772-6.
    • (2007) Neurol Res , vol.29 , pp. 772-776
    • Nagata, T.1    Nagano, I.2    Shiote, M.3    Narai, H.4    Murakami, T.5    Hayashi, T.6
  • 98
    • 0032485033 scopus 로고    scopus 로고
    • Cerebrospinal fluid interleukin 6 in amyotrophic lateral sclerosis: Immunological parameter and comparison with inflammatory and non-inflammatory central nervous system diseases
    • Sekizawa T, Openshaw H, Ohbo K, Sugamura K, Itoyama Y, Niland JC. Cerebrospinal fluid interleukin 6 in amyotrophic lateral sclerosis: immunological parameter and comparison with inflammatory and non-inflammatory central nervous system diseases. J Neurol Sci 1998;154:194-9.
    • (1998) J Neurol Sci , vol.154 , pp. 194-199
    • Sekizawa, T.1    Openshaw, H.2    Ohbo, K.3    Sugamura, K.4    Itoyama, Y.5    Niland, J.C.6
  • 99
    • 2442701519 scopus 로고    scopus 로고
    • Increased lipid peroxidation in sera of ALS patients: A potential biomarker of disease burden
    • Simpson EP, Henry YK, Henkel JS, Smith RG, Appel SH. Increased lipid peroxidation in sera of ALS patients: a potential biomarker of disease burden. Neurology 2004;62:1758-65.
    • (2004) Neurology , vol.62 , pp. 1758-1765
    • Simpson, E.P.1    Henry, Y.K.2    Henkel, J.S.3    Smith, R.G.4    Appel, S.H.5
  • 100
    • 0028178503 scopus 로고
    • Increased concentration of C4d complement protein in CSF in amyotrophic lateral sclerosis
    • Tsuboi Y, Yamada T. Increased concentration of C4d complement protein in CSF in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 1994;57:859-61.
    • (1994) J Neurol Neurosurg Psychiatry , vol.57 , pp. 859-861
    • Tsuboi, Y.1    Yamada, T.2
  • 101
    • 36248939088 scopus 로고    scopus 로고
    • Zetterberg H1 Jacobsson J, Rosengren L, Blennow K, Andersen PM. Cerebrospinal fluid neurofilament light levels in amyotrophic lateral sclerosis: impact of SOD1 genotype. Eur J Neurol 2007;14:1329-33.
    • Zetterberg H1 Jacobsson J, Rosengren L, Blennow K, Andersen PM. Cerebrospinal fluid neurofilament light levels in amyotrophic lateral sclerosis: impact of SOD1 genotype. Eur J Neurol 2007;14:1329-33.
  • 105
    • 0035575761 scopus 로고    scopus 로고
    • Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis associated mutations develop motor neuron disease
    • Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, et al. Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis associated mutations develop motor neuron disease. J Neurosci 2001;23:9246-54.
    • (2001) J Neurosci , vol.23 , pp. 9246-9254
    • Nagai, M.1    Aoki, M.2    Miyoshi, I.3    Kato, M.4    Pasinelli, P.5    Kasai, N.6
  • 106
    • 35348932113 scopus 로고    scopus 로고
    • Transgenic animal models of neurodegenerative diseases and their application to treatment development
    • Rockenstein E, Crews L, Masliah E. Transgenic animal models of neurodegenerative diseases and their application to treatment development. Adv Drug Deliv Rev 2007;59:1093-102.
    • (2007) Adv Drug Deliv Rev , vol.59 , pp. 1093-1102
    • Rockenstein, E.1    Crews, L.2    Masliah, E.3
  • 108
    • 29144536703 scopus 로고    scopus 로고
    • Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord
    • Ghadge GD, Wang L, Sharma K, Monti AL, Bindokas V, Stevens FJ, et al. Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord. Neurobiol Dis 2006;21:194-205.
    • (2006) Neurobiol Dis , vol.21 , pp. 194-205
    • Ghadge, G.D.1    Wang, L.2    Sharma, K.3    Monti, A.L.4    Bindokas, V.5    Stevens, F.J.6
  • 109
    • 0028284779 scopus 로고
    • Motor neuron degeneration in mice that express a human Cu/Zn superoxide dismutase mutation
    • Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, et al. Motor neuron degeneration in mice that express a human Cu/Zn superoxide dismutase mutation. Science 1994;264:1772-5.
    • (1994) Science , vol.264 , pp. 1772-1775
    • Gurney, M.E.1    Pu, H.2    Chiu, A.Y.3    Dal Canto, M.C.4    Polchow, C.Y.5    Alexander, D.D.6
  • 110
    • 0037137502 scopus 로고    scopus 로고
    • Differential vulnerability of cranial motor neurons in mouse models with motor neuron degeneration
    • Haenggeli C, Kato AC. Differential vulnerability of cranial motor neurons in mouse models with motor neuron degeneration. Neurosci Lett 2002;335:39-43.
    • (2002) Neurosci Lett , vol.335 , pp. 39-43
    • Haenggeli, C.1    Kato, A.C.2
  • 111
    • 0031886591 scopus 로고    scopus 로고
    • Selegiline is ineffective in a collaborative double-blind, placebo-controlled trial for treatment of amyotrophic lateral sclerosis
    • Lange DJ, Murphy PL, Diamond B, Appel V, Lai EC, Younger DS, et al. Selegiline is ineffective in a collaborative double-blind, placebo-controlled trial for treatment of amyotrophic lateral sclerosis. Arch Neurol 1998;55:93-6.
    • (1998) Arch Neurol , vol.55 , pp. 93-96
    • Lange, D.J.1    Murphy, P.L.2    Diamond, B.3    Appel, V.4    Lai, E.C.5    Younger, D.S.6
  • 112
    • 33644837764 scopus 로고    scopus 로고
    • Early signs of motor neuron vulnerability in a disease model system: Characterization of transverse slice cultures of spinal cord isolated from embryonic ALS mice
    • Avossa D, Grandolfo M, Mazzarol F, Zatta M, Ballerini L. Early signs of motor neuron vulnerability in a disease model system: characterization of transverse slice cultures of spinal cord isolated from embryonic ALS mice. Neuroscience 2006;138:1179-94.
    • (2006) Neuroscience , vol.138 , pp. 1179-1194
    • Avossa, D.1    Grandolfo, M.2    Mazzarol, F.3    Zatta, M.4    Ballerini, L.5
  • 113
    • 2442646665 scopus 로고    scopus 로고
    • A double-blind placebo-controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rhCNTF) in amyotrophic lateral sclerosis
    • ALS CNTF Treatment Study Group
    • ALS CNTF Treatment Study Group. A double-blind placebo-controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rhCNTF) in amyotrophic lateral sclerosis. Neurology 1996;46:1244-9.
    • (1996) Neurology , vol.46 , pp. 1244-1249
  • 114
    • 0034711749 scopus 로고    scopus 로고
    • A randomized controlled trial of recombinant Interferon beta-1a in ALS
    • Beghi E, Chio A, Inghilleri M, Mazzini L, Micheli A, Mora G, et al. A randomized controlled trial of recombinant Interferon beta-1a in ALS. Neurology 2000;54:469-74.
    • (2000) Neurology , vol.54 , pp. 469-474
    • Beghi, E.1    Chio, A.2    Inghilleri, M.3    Mazzini, L.4    Micheli, A.5    Mora, G.6
  • 115
    • 0031722582 scopus 로고    scopus 로고
    • European ALS/IGF-I Study Group. A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis
    • Borasio GD, Robberecht W, Leigh PN, Emile J, Guiloff RJ, Jerusalem F, et al; European ALS/IGF-I Study Group. A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. Neurology 1998;51:583-6.
    • (1998) Neurology , vol.51 , pp. 583-586
    • Borasio, G.D.1    Robberecht, W.2    Leigh, P.N.3    Emile, J.4    Guiloff, R.J.5    Jerusalem, F.6
  • 116
    • 3042835531 scopus 로고    scopus 로고
    • Tamoxifen[tri-phenylethylene protein kinase inhibitor] phase Ii dose-escalation clinical trial in ALS: Study design and safety results in first 50 patients
    • Brooks B, Sanjak M, Roelke KA, Parnell JP, Peper SM, Houdek AM, et al. Tamoxifen[tri-phenylethylene protein kinase inhibitor] phase Ii dose-escalation clinical trial in ALS: Study design and safety results in first 50 patients. Amyotroph Lateral Scler Other Motor Neuron Disord 2003;4(Suppl. 1):30.
    • (2003) Amyotroph Lateral Scler Other Motor Neuron Disord , vol.4 , Issue.SUPPL. 1 , pp. 30
    • Brooks, B.1    Sanjak, M.2    Roelke, K.A.3    Parnell, J.P.4    Peper, S.M.5    Houdek, A.M.6
  • 117
    • 10744222650 scopus 로고    scopus 로고
    • Northeast ALS Consortium. A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis
    • Cudkowicz M, Shefner J, Schoenfeld D, Brown RJ, Johnson H, Qureshi M, et al.; Northeast ALS Consortium. A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis. Neurol 2003;61:456-64.
    • (2003) Neurol , vol.61 , pp. 456-464
    • Cudkowicz, M.1    Shefner, J.2    Schoenfeld, D.3    Brown, R.J.4    Johnson, H.5    Qureshi, M.6
  • 123
    • 0029040463 scopus 로고
    • Randomized, double-blind, controlled trial of acetylcysteine in amyotrophic lateral sclerosis
    • Louwerse ES, Weverling GJ, Bossuyt PM, Meyjes FE, de Jong JM. Randomized, double-blind, controlled trial of acetylcysteine in amyotrophic lateral sclerosis. Arch Neurol 1995;52: 559-64.
    • (1995) Arch Neurol , vol.52 , pp. 559-564
    • Louwerse, E.S.1    Weverling, G.J.2    Bossuyt, P.M.3    Meyjes, F.E.4    de Jong, J.M.5
  • 124
    • 33645802414 scopus 로고    scopus 로고
    • Pentoxifylline European Group. Pentoxifylline in ALS: A double-blind, randomized, multicenter, placebo-controlled trial
    • Meininger V, Asselain B, Guillet P, Leigh PN, Ludolph A, Lacomblez L, et al.; Pentoxifylline European Group. Pentoxifylline in ALS: a double-blind, randomized, multicenter, placebo-controlled trial. Neurology 2006;66:88-92.
    • (2006) Neurology , vol.66 , pp. 88-92
    • Meininger, V.1    Asselain, B.2    Guillet, P.3    Leigh, P.N.4    Ludolph, A.5    Lacomblez, L.6
  • 126
    • 10544254257 scopus 로고    scopus 로고
    • WALS Study Group. Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis
    • Miller R, Moore DH, Young L, Armon C, Barohn RJ, Bromberg MB, et al.; WALS Study Group. Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis. Neurology 1996;47:1383-8.
    • (1996) Neurology , vol.47 , pp. 1383-1388
    • Miller, R.1    Moore, D.H.2    Young, L.3    Armon, C.4    Barohn, R.J.5    Bromberg, M.B.6
  • 127
    • 0035836649 scopus 로고    scopus 로고
    • Western ALS Study Group. Phase III randomized trial of gabapentin in patients with amyotrophic lateral sclerosis
    • Miller RG, Moore DH, Gelinas DF, Dronsky B, Mendoza M, Barohn R, et al.; Western ALS Study Group. Phase III randomized trial of gabapentin in patients with amyotrophic lateral sclerosis. Neurology 2001;56:843-8.
    • (2001) Neurology , vol.56 , pp. 843-848
    • Miller, R.G.1    Moore, D.H.2    Gelinas, D.F.3    Dronsky, B.4    Mendoza, M.5    Barohn, R.6
  • 128
    • 9044219848 scopus 로고    scopus 로고
    • A placebo-controlled trial of recombinant human ciliary neurotrophic (rhCNTF) factor in amyotrophic lateral sclerosis
    • Miller RG, Petajan JH, Bryan WW, Armon C, Barohn RJ, Goodpasture JC, et al. A placebo-controlled trial of recombinant human ciliary neurotrophic (rhCNTF) factor in amyotrophic lateral sclerosis. Ann Neurol 1996;39:256-60.
    • (1996) Ann Neurol , vol.39 , pp. 256-260
    • Miller, R.G.1    Petajan, J.H.2    Bryan, W.W.3    Armon, C.4    Barohn, R.J.5    Goodpasture, J.C.6
  • 131
    • 0026729250 scopus 로고
    • Intrathecal thyrotropin-releasing hormone does not alter the progressive course of ALS: Experience with an intrathecal drug delivery system
    • Munsat TL, Taft J, Jackson IM, Andres PL, Hollander D, Skerry L, et al. Intrathecal thyrotropin-releasing hormone does not alter the progressive course of ALS: experience with an intrathecal drug delivery system. Neurology 1992;42:1049-53.
    • (1992) Neurology , vol.42 , pp. 1049-1053
    • Munsat, T.L.1    Taft, J.2    Jackson, I.M.3    Andres, P.L.4    Hollander, D.5    Skerry, L.6
  • 132
    • 0038723463 scopus 로고    scopus 로고
    • A double-blind randomized clinical trial in amyotrophic lateral sclerosis using lamotrigine: Effects on CSF glutamate, aspartate, branched-chain amino acid levels and clinical parameters
    • Ryberg H, Askmark H, Persson LI. A double-blind randomized clinical trial in amyotrophic lateral sclerosis using lamotrigine: effects on CSF glutamate, aspartate, branched-chain amino acid levels and clinical parameters. Acta Neurol Scand 2003; 108:1-8.
    • (2003) Acta Neurol Scand , vol.108 , pp. 1-8
    • Ryberg, H.1    Askmark, H.2    Persson, L.I.3
  • 134
    • 0029661207 scopus 로고    scopus 로고
    • Tandan R, Bromberg MB, Forshew D, Fries TJ, Badger GJ, Carpenter J, et al. A controlled trial of amino acid therapy in amyotrophic lateral sclerosis: I. clinical, functional, and maximum isometric torque data. Neurology 1996;47:1220-6.
    • Tandan R, Bromberg MB, Forshew D, Fries TJ, Badger GJ, Carpenter J, et al. A controlled trial of amino acid therapy in amyotrophic lateral sclerosis: I. clinical, functional, and maximum isometric torque data. Neurology 1996;47:1220-6.
  • 135
    • 0344076149 scopus 로고    scopus 로고
    • A controlled trial of recombinant methionyl human BDNF in ALS
    • The BDNF Study Group
    • The BDNF Study Group. A controlled trial of recombinant methionyl human BDNF in ALS. Neurology 1999;52:1427-33.
    • (1999) Neurology , vol.52 , pp. 1427-1433
  • 136
    • 0027763486 scopus 로고
    • Branched-chain amino acids and amyotrophic lateral sclerosis: A treatment failure?
    • The Italian ALS Study Group
    • The Italian ALS Study Group. Branched-chain amino acids and amyotrophic lateral sclerosis: a treatment failure? Neurology 1993;43:2466-70.
    • (1993) Neurology , vol.43 , pp. 2466-2470
  • 138
  • 139
    • 33744786331 scopus 로고    scopus 로고
    • Preclinical trials-an update on translational research in ALS
    • Ludolph AC, Sperfeld AD. Preclinical trials-an update on translational research in ALS. Neurodegener Dis 2005;2:215-9.
    • (2005) Neurodegener Dis , vol.2 , pp. 215-219
    • Ludolph, A.C.1    Sperfeld, A.D.2
  • 143
    • 55849137105 scopus 로고    scopus 로고
    • Design of phase II ALS clinical trials
    • Schoenfeld D, Cudkowicz M. Design of phase II ALS clinical trials. Amyotroph Lateral Scler 2007;8(Suppl. 1):60-1.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 60-61
    • Schoenfeld, D.1    Cudkowicz, M.2
  • 144
    • 36148996513 scopus 로고    scopus 로고
    • Swash M. Learning from failed trials in ALS. Lancet Neurol 2007;6.1034-5.
    • Swash M. Learning from failed trials in ALS. Lancet Neurol 2007;6.1034-5.
  • 145
    • 47249134106 scopus 로고    scopus 로고
    • Design and implementation of a multi-phase, adaptive-design clinical trial for subjects with ALS
    • Swartz A, Shefner J, Yu H, Thornell B, Sherman A, Cudkowicz M. Design and implementation of a multi-phase, adaptive-design clinical trial for subjects with ALS. Amyotrophic Lateral Sclerosis 2007;8(Suppl. 1):142-3.
    • (2007) Amyotrophic Lateral Sclerosis , vol.8 , Issue.SUPPL. 1 , pp. 142-143
    • Swartz, A.1    Shefner, J.2    Yu, H.3    Thornell, B.4    Sherman, A.5    Cudkowicz, M.6
  • 147
    • 55849100214 scopus 로고    scopus 로고
    • NEALS Consortium. Assessment of the clinical significance in the change of decline in ALSFRS-R
    • Grasso D, Simpson E, Tellier J, Barber J, Shefner J, Cudkowicz M; NEALS Consortium. Assessment of the clinical significance in the change of decline in ALSFRS-R. Amyotroph Lateral Scler 2007;8(Suppl. 1):140-1.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 140-141
    • Grasso, D.1    Simpson, E.2    Tellier, J.3    Barber, J.4    Shefner, J.5    Cudkowicz, M.6
  • 149
    • 0035852960 scopus 로고    scopus 로고
    • Treatment of ALS with pleconaril
    • Ansevin CF. Treatment of ALS with pleconaril. Neurology 2001;56:691-2.
    • (2001) Neurology , vol.56 , pp. 691-692
    • Ansevin, C.F.1
  • 150
    • 33845418269 scopus 로고    scopus 로고
    • Selecting promising ALS therapies in clinical trials
    • Cheung YK, Gordon PH, Levin B. Selecting promising ALS therapies in clinical trials. Neurology 2006;67:1748-51.
    • (2006) Neurology , vol.67 , pp. 1748-1751
    • Cheung, Y.K.1    Gordon, P.H.2    Levin, B.3
  • 151
    • 55849088921 scopus 로고    scopus 로고
    • th International Symposium on ALS/MND. Yokohama, Japan. 30 Nov-2 Dec 2006. Clinical Work in Progress and Care Practice and Scientific Work in Progress. Abstract CW187, 10.
    • th International Symposium on ALS/MND. Yokohama, Japan. 30 Nov-2 Dec 2006. Clinical Work in Progress and Care Practice and Scientific Work in Progress. Abstract CW187, 10.
  • 153
  • 154
    • 48249096561 scopus 로고    scopus 로고
    • TRO19622 is well tolerated and target plasma concentrations are obtained at doses 250/500 mg once a day in a one month ALS phase 1b, add-on to riluzole, clinical study
    • Verschueren A, Lacomblez L, Abitbol J-L, Cuvier V, Jouve E, Blin O, et al. TRO19622 is well tolerated and target plasma concentrations are obtained at doses 250/500 mg once a day in a one month ALS phase 1b, add-on to riluzole, clinical study. Amyotroph Lateral Scler 2007;8(Suppl. 1):59-60.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 59-60
    • Verschueren, A.1    Lacomblez, L.2    Abitbol, J.-L.3    Cuvier, V.4    Jouve, E.5    Blin, O.6
  • 155
    • 55849085247 scopus 로고    scopus 로고
    • Double-blind, placebo controlled safety study of ritonavir and hydroxyurea in patients with ALS
    • Lomen-Hoerth C, Squire L, Scott S, McCarthy J, Olney R. Double-blind, placebo controlled safety study of ritonavir and hydroxyurea in patients with ALS. Amyotroph Lateral Scler 2007;8(Suppl. 1):60.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 60
    • Lomen-Hoerth, C.1    Squire, L.2    Scott, S.3    McCarthy, J.4    Olney, R.5
  • 157
    • 44349094126 scopus 로고    scopus 로고
    • Evaluation of treatment with Rasagiline in patients with Amyotrophic Lateral Sclerosis
    • Drory V, Artmonov I, Nefussy B. Evaluation of treatment with Rasagiline in patients with Amyotrophic Lateral Sclerosis. Amyotroph Lateral Scler 2007;8(Suppl. 1):143-4.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 143-144
    • Drory, V.1    Artmonov, I.2    Nefussy, B.3
  • 160
    • 55849091207 scopus 로고    scopus 로고
    • The long-term effect of edaravone in ALS patients
    • Nakagawa N, Ohkubo H, Yoshino H. The long-term effect of edaravone in ALS patients. Amyotroph Lateral Scler 2007;8 (Suppl. 1):146.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 146
    • Nakagawa, N.1    Ohkubo, H.2    Yoshino, H.3
  • 162
    • 55849097536 scopus 로고    scopus 로고
    • A pilot trial of levetiracetam for cramps, spasticity and neuroprotection in patients with motor neuron diseases
    • Bedlack R, Hawes J, Pastula D, Heydt D, Burke P. A pilot trial of levetiracetam for cramps, spasticity and neuroprotection in patients with motor neuron diseases. Amyotroph Lateral Scler 2007;8(Suppl. 1):148.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 148
    • Bedlack, R.1    Hawes, J.2    Pastula, D.3    Heydt, D.4    Burke, P.5
  • 163
    • 55849122134 scopus 로고    scopus 로고
    • Pyrimethamine as a therapy for SOD1 associated FALS: Early findings
    • Lange D, Seidel G, Benjamin D, Scott S, Vieira F. Pyrimethamine as a therapy for SOD1 associated FALS: early findings. Amyotroph Lateral Scler 2007;8(Suppl. 1):148-9.
    • (2007) Amyotroph Lateral Scler , vol.8 , Issue.SUPPL. 1 , pp. 148-149
    • Lange, D.1    Seidel, G.2    Benjamin, D.3    Scott, S.4    Vieira, F.5
  • 164
  • 165
    • 55849130568 scopus 로고    scopus 로고
    • Study Group AVP-9233. Dextromethorphan-quinidine (DMQ) treatment of pseudobulbar affect (PBA) in Amyotrophic Lateral Sclerosis (ALS)
    • Brooks BR, Bradley WG, Arnold R, Pope LE, Berg JE, Olney RK, et al.; Study Group AVP-9233. Dextromethorphan-quinidine (DMQ) treatment of pseudobulbar affect (PBA) in Amyotrophic Lateral Sclerosis (ALS). Amyotroph Lateral Scler 2006;7(Suppl. 1): 110.
    • (2006) Amyotroph Lateral Scler , vol.7 , Issue.SUPPL. 1 , pp. 110
    • Brooks, B.R.1    Bradley, W.G.2    Arnold, R.3    Pope, L.E.4    Berg, J.E.5    Olney, R.K.6
  • 166
    • 34548278481 scopus 로고    scopus 로고
    • Safety and pharmacokinetics of repeated doses of TRO19622, a drug candidate for the treatment of amyotrophic lateral sclerosis and spinal muscular atrophy
    • Abitbol JL, Cuvier V, Bordet T, Drouot C, Berna P, Pruss R. Safety and pharmacokinetics of repeated doses of TRO19622, a drug candidate for the treatment of amyotrophic lateral sclerosis and spinal muscular atrophy. Amyotroph Lateral Scler 2006;7(Suppl. 1):112-3.
    • (2006) Amyotroph Lateral Scler , vol.7 , Issue.SUPPL. 1 , pp. 112-113
    • Abitbol, J.L.1    Cuvier, V.2    Bordet, T.3    Drouot, C.4    Berna, P.5    Pruss, R.6
  • 167
    • 34250767883 scopus 로고    scopus 로고
    • A multicentre, dose ranging safety and pharmacokinetics study of arimoclomol in ALS
    • Cudkowicz M, Shefner J, Simpson E, Grasso D, Sherman A, Yu H, et al. A multicentre, dose ranging safety and pharmacokinetics study of arimoclomol in ALS. Amyotroph Lateral Scler 2006; 7(Suppl. 1):113.
    • (2006) Amyotroph Lateral Scler , vol.7 , Issue.SUPPL. 1 , pp. 113
    • Cudkowicz, M.1    Shefner, J.2    Simpson, E.3    Grasso, D.4    Sherman, A.5    Yu, H.6
  • 168
    • 55849099847 scopus 로고    scopus 로고
    • QALS Study Group. Is a phase III trial of coenzyme Q10 (CoQ10) for ALS justified? Results of the phase II randomized controlled trial of CoQ10 for ALS
    • Kaufmann P, Thompson JL, Levy G, Barsdorf AI, Buchsbaum R, Montes J, et al.; QALS Study Group. Is a phase III trial of coenzyme Q10 (CoQ10) for ALS justified? Results of the phase II randomized controlled trial of CoQ10 for ALS. Neurology 2008;70(Suppl. 1)A238.
    • (2008) Neurology , vol.70 , Issue.SUPPL. 1
    • Kaufmann, P.1    Thompson, J.L.2    Levy, G.3    Barsdorf, A.I.4    Buchsbaum, R.5    Montes, J.6
  • 169
    • 55849139653 scopus 로고    scopus 로고
    • A phase I-II multicenter study on G-CSF in amyotrophic lateral sclerosis (STEMALS)
    • Chiò A, Mora G, Caponnetto C, Siciliano G, Sabatelli M, La Bella V, et al. A phase I-II multicenter study on G-CSF in amyotrophic lateral sclerosis (STEMALS). Neurology 2008;70(Suppl. 1): A192.
    • (2008) Neurology , vol.70 , Issue.SUPPL. 1
    • Chiò, A.1    Mora, G.2    Caponnetto, C.3    Siciliano, G.4    Sabatelli, M.5    La Bella, V.6


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