Strategies for clinical approach to neurodegeneration in amyotrophic lateral sclerosis

Archives Italiennes de Biologie

Volumn 149, Issue 1, 2011, Pages 151-167

  Carlesi, C ^a   Pasquali, L ^a   Piazza, S ^a   Lo Gerfo, A ^a   Caldarazzo Ienco, E ^a   Alessi, R ^a   Fornai, F ^a,b   Siciliano, G ^a  

^a UNIVERSITY OF PISA   (Italy)

^b IRCCS NEUROMED   (Italy)

Author keywords

Amyotrophic lateral sclerosis; Clinical trial; Therapeutic strategies

Indexed keywords

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL; ARTICLE; HUMAN; NERVE DEGENERATION;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; HUMANS; NERVE DEGENERATION;

EID: 79952848269     PISSN: 00039829     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (153)

1. Abe K., Pan L.H., Watanabe M., Kato T., Itoyama Y. Induction of nitrotyrosine-like immunoreactivity in the lower motor neuron of amyotrophic lateral sclerosis. Neurosci. Lett., 199: 152-154, 1995.
2. Abe K., Pan L.H., Watanabe M., Konno H., Kato T., Itoyama Y. Upregulation of protein-tyrosine nitration in the anterior horn cells of amyotrophic lateral sclerosis. Neurol. Res., 19: 124-128, 1997. (Pubitemid 27190700)
3. Aggarwal S.P., Zinman L., Simpson E., McKinley J., Jackson K.E., Pinto H., Kaufman P., Conwit R.A., Schoenfeld D., Shefner J., Cudkowicz M. Northeast and Canadian Amyotrophic Lateral Sclerosis consortia. Safety and efficacy of lithium in combination with riluzole for treatment of amyotrophic lateral sclerosis: a randomised, doubleblind, placebo-controlled trial. Lancet Neurol., 9: 481-488, 2010.
4. Aitkens S.G., McCrory M.A., Kilmer D.D., Bernauer E.M. Moderate resistance exercise program: its effect in slowly progressive neuromuscular disease. Arch. Phys. Med. Rehabil., 74: 711-715, 1993. (Pubitemid 23203547)
5. Albo F., Pieri M., Zona C. Modulation of AMPA receptors in spinal motor neurons by the neuroprotective agent riluzole. J. Neurosci. Res., 78: 200-207, 2004. (Pubitemid 39313792)
6. Allison A.C., Cacabelos R., Lombardi V.R., Alvarez X.A., Vigo C. Celastrol, a potent antioxidant and anti-inflammatory drug, as a possible treatment for Alzheimer's disease. Prog. Neuropsychopharmacol. Biol. Psychiatry, 25: 1341-1357, 2001. (Pubitemid 33022713)
7. Anderson C.M. and Swanson R.A. Astrocyte glutamate transport: review of properties, regulation, and physiological functions. Glia, 32: 1-14, 2000.
8. Appel S.H. and Simpson E.P. Activated microglia: the silent executioner in neurodegenerative disease? Curr. Neurol. Neurosci. Rep., 1: 303-305, 2001.
9. Azzouz M., Ralph G.S., Storkebaum E., Walmsley L.E., Mitrophanous K.A., Kingsman S.M., Carmeliet P., Mazarakis N.D. VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model. Nature, 429: 413-417, 2004. (Pubitemid 38715132)
10. Beal M.F. Oxidatively modified proteins in aging and disease. Free Radical Biol. Med., 32: 797-803, 2002. (Pubitemid 34439250)
11. Beal M.F., Ferrante R.J., Browne S.E., Matthews R.T., Kowall N.W., Brown Jr. R.H. Increased 3-nitroty-rosine in both sporadic and familial amyotrophic lateral sclerosis. Ann. Neurol., 42: 644-654, 1997. (Pubitemid 27439028)
12. Beckman J.S., Estevez A.G., Crow J.P., Barbeito L. Superoxide dismutase and the death of motoneurons in ALS. Trends Neurosci., 24: S15-S20, 2001.
13. Bello-Haas, V.D., Florence, J.M., Kloos, A.D., Scheirbecker, J., Lopate, G., Hayes, S.M., Pioro, E.P., Mitsumoto, H. A randomized controlled trial of resistance exercise in individuals with ALS. Neurology, 68: 2003-2007, 2007. (Pubitemid 46869704)
14. Bensimon G., Lacomblez L., Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N. Engl. J. Med., 330: 585-591, 1994. (Pubitemid 24065791)
15. Berger Z., Ravikumar B., Menzies F.M., Oroz L.G., Underwood B.R., Pangalos M.N., Schmitt I., Wullner U., Evert B.O., O'Kane C.J., Rubinsztein D.C. Rapamycin alleviates toxicity of different aggregate-prone proteins. Hum. Mol. Genet., 15: 433-442, 2006. (Pubitemid 43159895)
16. Berridge M.J., Downes C.P., Hanley M.R. Neural and developmental actions of lithium: A unifying hypothesis. Cell, 59: 411-419, 1989. (Pubitemid 19284456)
17. Borasio G.D., Robberecht W., Leigh P.N., Emile J., Guiloff R.J., Jerusalem F., Silani V., Vos P.E., Wokke J.H., Dobbins T. A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. European ALS/IGF-I Study Group. Neurology, 51: 583-586, 1998. (Pubitemid 28406343)
18. Bossy-Wetzel E., Schwarzenbacher R., Lipton S.A. Molecular pathways to neurodegeneration. Nat. Med., 10 Suppl: S2-9, 2004. (Pubitemid 38901861)
19. Bozik M., Ingersoll E., Voiles L., Mather J., Amburgey C., Moritz J., Archibald D., Sullivan M., Gribkoff V., Miller R., Mitsumoto H., Moore D., Schoenfeld D., Shefner J., Cudkowicz M. KNS-760704-CL201, Part 1: A 12-Week Phase 2 Study of the Safety, Tolerability, and Clinical Effects of KNS 760704 in ALS Subjects. In: 20th International Symposium on ALS/MND, Berlin, Germany, 8-10 December; Clinical Trials and Trial Design, 2009.
20. Brockington A., Wharton S.B., Fernando M., Gelsthorpe C.H., Baxter L., Ince P.G., Lewis C.E., Shaw P.J. Expression of vascular endothe-lial growth factor and its receptors in the central nervous system in amyotrophic lateral sclerosis. J. Neuropathol. Exp. Neurol., 65: 26-36, 2006.
21. Brooks B.R., Miller R.G., Swash M., Munsat T.L. World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph. Lateral Scler. Other Motor Neuron Disord., 1: 293-299, 2000.
22. Bruijn L.I., Miller T.M., Cleveland D.W. Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu. Rev. Neurosci., 27: 723-749, 2004. (Pubitemid 39050419)
23. Cairns N.J., Lee V.M., Trojanowski J.Q. The cytoskeleton in neurodegenerative diseases. J. Pathol., 204: 438-449, 2004. (Pubitemid 39468155)
24. Calderó J., Brunet N., Tarabal O., Piedrafita L., Hereu M., Ayala V., Esquerda J.E. Lithium prevents excitotoxic cell death of motoneurons in organotypic slice cultures of spinal cord. Neuroscience, 165: 1353-1369, 2010.
25. Carelli V., Liguori R., Cordivari C., Bianchedi G., Montagna P. Ceftriaxone is ineffective in ALS. Ital. J. Neurol. Sci., 15: 66, 1994. (Pubitemid 24101429)
26. Carod Artal F.J., Perez Lopez-Fraile I., Gracia Naya M., Giron Mombiela J.A. Failure of empirical treatment with ceftriaxone in motor neuron disease. Neurologia, 9: 29-31, 1994. (Pubitemid 24088263)
27. Carreras I., Yuruker S., Aytan N., Hossain L., Choi J.K., Jenkins B.G., Kowall N.W., Dedeoglu A. Moderate exercise delays the motor performance decline in a transgenic model of ALS. Brain Res., 1313: 192-201, 2010.
28. Carriedo S.G, Yin H.Z., Weiss J.H. Motor neurons are selectively vulnerable to AMPA/kainate receptor-mediated injury in vitro. J. Neurosci., 16: 4069-4079, 1996. (Pubitemid 26192403)
29. Castro R.E., Solá; S., Ramalho R.M., Steer C.J., Rodrigues C.M. The bile acid tauroursodeoxycholic acid modulates phosphorylation and translocation of bad via phosphatidylinositol 3-kinase in glutamate-induced apoptosis of rat cortical neurons. J. Pharmacol. Exp. Ther., 311: 845-852, 2004. (Pubitemid 39391575)
30. Chen H.S, Pellegrini J.W., Aggarwal S.K., Lei S.Z., Warach S., Jensen F.E., Lipton S.A. Open-channel block of N-methyl-D-aspartate (NMDA) responses by memantine: therapeutic advantage against NMDA receptor-mediated neurotoxicity. J. Neurosci., 12: 4427-4436, 1992.
31. Chen H.S. and Lipton S.A. The chemical biology of clinically tolerated NMDA receptor antagonists. J. Neurochem., 97: 1611-1626, 2006. (Pubitemid 43873656)
32. Chiò A., Benzi G., Dossena M., Mutani R., Mora, G. Severely increased risk of amyotrophic lateral sclerosis among Italian professional football players. Brain, 128: 472-476, 2005. (Pubitemid 40380183)
33. Choi D.W. Glutamate neurotoxicity and diseases of the nervous system. Neuron, 1: 623-634, 1988.
34. Cleveland D.W. From Charcot to SOD1: mechanisms of selective motor neuron death in ALS. Neuron, 24: 515-520, 1999.
35. Couratier P., Vallat J.M., Merle L., Preux P.M., Hugon J. Report of six sporadic cases of ALS patients receiving ceftriaxone. Therapie, 49: 146, 1994. (Pubitemid 24332241)
36. Crews L., Spencer B., Desplats P., Patrick C., Paulino A., Rockenstein E., Hansen L., Adame A., Galasko D., Masliah E. Selective molecular alterations in the autophagy pathway in patients with Lewy body disease and in models of alpha-synucleinopathy. PLoS One, 5: e9313, 2010.
37. Crippa V., Sau D., Rusmini P., Boncoraglio A., Onesto E., Bolzoni E., Galbiati M., Fontana E., Marino M., Carra S., Bendotti C., De Biasi S., Poletti A. The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS). Hum. Mol. Genet., 19: 3440-3456, 2010.
38. Crow J.P., Calingasan N.Y., Chen J., Hill J.L., Beal M.F. Manganese porphyrin given at symptom onset markedly extends survival of ALS mice. Ann. Neurol., 58: 258-265, 2005. (Pubitemid 41098883)
39. Cudkowicz M.E., Shefner J.M., Simpson E., Grasso D., Yu H., Zhang H., Shui A., Schoenfeld D., Brown R.H., Wieland S., Barber J.R., Northeast ALS Consortium. Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis. Muscle Nerve, 38: 837-844, 2008. (Pubitemid 351915317)
40. de Carvalho M., Pinto S., Costa J., Evangelista T., Ohana B., Pinto A. A randomized, placebo-controlled trial of memantine for functional disability in amyotrophic lateral sclerosis. Amyotroph. Lateral Scler., 11: 456-460, 2010
41. Di Lazzaro V., Dileone M., Pilato F., Profice P., Cioni B., Meglio M., Papacci F., Sabatelli M., Musumeci G., Ranieri F., Tonali P.A. Long-term motor cortex stimulation for amyotrophic lateral sclerosis. Brain Stimul., 3: 22-27, 2010.
42. Di Lazzaro V., Dileone M., Pilato F., Profice P., Ranieri F., Musumeci G., Angelucci F., Sabatelli M., Tonali P.A. Repetitive transcranial magnetic stimulation for ALS. A preliminary controlled study. Neurosci. Lett., 408: 135-140, 2006. (Pubitemid 44498953)
43. Doble A. The pharmacology and mechanism of action of riluzole. Neurology, 47: S233-241, 1996. (Pubitemid 26422576)
44. Dobrowolny G., Giacinti C., Pelosi L., Nicoletti C., Winn N., Barberi L., Molinaro M., Rosenthal N., Musaró A. Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model. J. Cell Biol., 168: 193-199, 2005. (Pubitemid 40248217)
45. Drory V.E., Goltsman E., Reznik J.G., Mosek A., Korczyn A.D. The value of muscle exercise in patients with amyotrophic lateral sclerosis. J. Neurol. Sci., 191: 133-137, 2001. (Pubitemid 33035906)
46. Edaravone Acute Infarction Study Group. Effect of a novel free radical scavenger, edaravone (MCI-186), on acute brain infarction. Randomized, placebo-controlled, double-blind study at multicenters. Cerebrovasc. Dis., 15: 222-229, 2003.
47. Feng H.L., Leng Y., Ma C.H., Zhang J., Ren M., Chuang D.M. Combined lithium and valproate treatment delays disease onset, reduces neurological deficits and prolongs survival in an amyotrophic lateral sclerosis mouse model. Neuroscience, 155: 567-572, 2008.
48. Ferrante R.J., Browne S.E., Shinobu L.A., Bowling A.C., Baik M.J, MacGarvey U., Kowall N.W., Brown Jr. R.H., Beal M.F. Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis. J. Neurochem., 69: 2064-2074, 1997. (Pubitemid 27452754)
49. Ferrucci M., Spalloni A., Bartalucci A., Cantafora E., Fulceri F., Nutini M., Longone P., Paparelli A., Fornai F. A systematic study of brainstem motor nuclei in a mouse model of ALS, the effects of lithium. Neurobiol. Dis., 37: 370-383, 2010.
50. Fitzmaurice P.S., Shaw I.C., Kleiner H.E., Miller R.T., Monks T.J., Lau S.S., Mitchell J.D., Lynch P.G. Evidence for DNA damage in amyotrophic lateral sclerosis. Muscle Nerve, 19: 797-798, 1996.
51. Fornai F., Longone P., Cafaro L., Kastsiuchenka O., Ferrucci M., Manca M.L., Lazzeri G., Spalloni A., Bellio N., Lenzi P., Modugno N., Siciliano G., Isidoro C., Murri L., Ruggieri S., Paparelli A. Lithium delays progression of amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. U.S.A., 105: 2052-2057, 2008. (Pubitemid 351439463)
52. Francis K., Bach J.R., DeLisa J.A. Evaluation and rehabilitation of patients with adult motor neuron disease. Arch. Phys. Med. Rehabil., 80: 951-963, 1999. (Pubitemid 29382421)
53. Franks M.E., Macpherson G.R., Figg W.D. Thalidomide. Lancet, 363, 1802-1811, 2004. (Pubitemid 38698386)
54. Gardiner P., Dai Y., Heckman C.J. Effects of exercise training on alpha-motoneurons. J. Appl. Physiol., 101: 1228-1236, 2006.
55. Genova M.L., Pich M.M., Bernacchia A., Bianchi C., Biondi A., Bovina C., Falasca A.I., Formiggini G., Castelli G.P., Lenaz G. The mitochondrial production of reactive oxygen species in relation to aging and pathology. Ann. N.Y. Acad. Sci., 1011: 86-100, 2004. (Pubitemid 38608395)
56. Gil Llano J.R. and Casado Naranjo I. Lack of improvement of motoneuron disease with ceftriaxone. Neurologia, 9: 205-206, 1994. (Pubitemid 24180294)
57. Gill A., Kidd J., Vieira F., Thompson K., Perrin S. No benefit from chronic lithium dosing in a siblingmatched, gender balanced, investigator-blinded trial using a standard mouse model of familial ALS. PLoS One, 4: e6489, 2009.
58. Gordon P.H., Moore D.H., Miller R.G., Florence J.M., Verheijde J.L., Doorish C., Hilton J.F., Spitalny G.M., MacArthur R.B., Mitsumoto H., Neville H.E., Boylan K., Mozaffar T., Belsh J.M., Ravits J., Bedlack R.S., Graves M.C., McCluskey L.F., Barohn R.J., Tandan R., Western ALS Study Group. Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial. Lancet Neurol., 6: 1045-1053, 2007. (Pubitemid 350116691)
59. Harvey W.T. and Martz D. Motor neuron disease recovery associated with IV ceftriaxone and antibabesia therapy. Acta Neurol. Scand., 115: 129-131, 2007. (Pubitemid 46018550)
60. Hassel B., Iversen E.G., Gjerstad L., Tauboll E. Up-regulation of hippocampal glutamate transport during chronic treatment with sodium valproate. J. Neurochem., 77: 1285-1292, 2001. (Pubitemid 32523681)
61. Hirano A. Cytopathology in amyotrophic lateral sclerosis. Adv. Neurol., 56: 91-101, 1991.
62. Holloszy J.O., Rennie M.J., Hickson R.C., Conlee R.K., Hagberg J.M. Physiological consequences of the biochemical adaptation to endurance exercise. Ann. N.Y. Acad. Sci., 301: 440-450, 1977. (Pubitemid 8226235)
63. Ihara Y., Nobukuni K., Takata H., Hayabara T. Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu,Zn-superoxide dismutase mutation. Neurol. Res., 27: 105-108, 2005. (Pubitemid 40189615)
64. Ito H., Wate R., Zhang J., Ohnishi S., Kaneko S., Ito H., Nakano S., Kusaka H. Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice. Exp. Neurol., 213: 448-455, 2008.
65. Jin K.L., Mao X.O., Greenberg D.A. Vascular endothelial growth factor: direct neuroprotective effect in in vitro ischemia. Proc. Natl. Acad. Sci. U.S.A., 97: 10242-10247, 2000.
66. Jin K., Zhu Y., Sun Y., Mao X.O., Xie L., Greenberg D.A. Vascular endothelial growth factor (VEGF) stimulates neurogenesis in vitro and in vivo. Proc. Natl. Acad. Sci. U.S.A., 99: 11946-11950, 2002. (Pubitemid 34994504)
67. Joo I.S., Hwang D.H., Seok J.I., Shin S.K., Kim S.U. Oral administration of memantine prolongs survival in a transgenic mouse model of amyotrophic lateral sclerosis. J. Clin. Neurol., 3: 181-186, 2007.
68. Kalmar B., Novoselov S., Gray A., Cheetham M.E., Margulis B., Greensmith L. Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1 mouse model of ALS. J. Neurochem., 107: 339-350, 2008.
69. Kanai H., Sawa A., Chen R.W., Leeds P., Chuang D.M. Valproic acid inhibits histone deacetylase activity and suppresses excitotoxicity-induced GAPDH nuclear accumulation and apoptotic death in neurons. Pharmacogenomics J., 4: 336-344, 2004. (Pubitemid 39341573)
70. Kaspar B.K., Lladó J., Sherkat N., Rothstein J.D., Gage F.H. Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model. Science, 301: 839-842, 2003. (Pubitemid 36962626)
71. Kaufmann P., Thompson J.L., Levy G., Buchsbaum R., Shefner J., Krivickas L.S., Katz J., Rollins Y., Barohn R.J., Jackson C.E., Tiryaki E., Lomen-Hoerth C., Armon C., Tandan R., Rudnicki S.A., Rezania K., Sufit R., Pestronk A., Novella S.P., Heiman-Patterson T., Kasarskis E.J., Pioro E.P., Montes J., Arbing R., Vecchio D., Barsdorf A., Mitsumoto H., Levin B., QALS Study Group. Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III. Ann. Neurol., 66: 235-244, 2009.
72. Kernochan L.E., Russo M.L., Woodling N.S., Huynh T.N., Avila A.M., Fischbeck K.H., Sumner C.J. The role of histone acetylation in SMN gene expression. Hum. Mol. Genet., 14: 1171-1182, 2005. (Pubitemid 40613906)
73. Kiaei M., Petri S., Kipiani K., Gardian G., Choi D.K., Chen J., Calingasan N.Y., Schafer P., Muller G.W., Stewart C., Hensley K., Beal M.F. Thalidomide and lenalidomide extend survival in a transgenic mouse model of amyotrophic lateral sclerosis. J. Neurosci., 26, 2467-2473, 2006. (Pubitemid 44699007)
74. Kieran D., Kalmar B., Dick J.R., Riddoch-Contreras J., Burnstock G., Greensmith L. Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat. Med., 10: 402-405, 2004. (Pubitemid 38508519)
75. Kipiani K., Kiaei M., Chen J., Calingasan N.Y., Beal M.F. Celastrol blocks motor neuron cell death and extends life in transgenic mouse model of amyotrophic lateral sclerosis. J. Neurochem., 90 (Suppl 1): 92, 2004.
76. Klug D., Rabani J., Fridovich I. A direct demonstration of the catalytic action of superoxide dismutase through the use of pulse radiolysis. J. Biol. Chem., 247: 4839-4842, 1972.
77. Kriz J., Nguyen M., Julien J. Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol. Dis., 10: 268-278, 2002. (Pubitemid 35217580)
78. Lai E.C., Felice K.J., Festoff B.W., Gawel M.J., Gelinas D.F., Kratz R., Murphy M.F., Natter H.M., Norris F.H., Rudnicki S.A. 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, 49: 1621-1630, 1997. (Pubitemid 28015128)
79. Lambrechts D., Storkebaum E., Morimoto M., Del-Favero J., Desmet F., Marklund S.L., Wyns S., Thijs V., Andersson J., van Marion I., Al-Chalabi A., Bornes S., Musson R., Hansen V., Beckman L., Adolfsson R., Pall H.S., Prats H., Vermeire S., Rutgeerts P., Katayama S., Awata T., Leigh N., Lang-Lazdunski L., Dewerchin M., Shaw C., Moons L., Vlietinck R., Morrison K.E., Robberecht W., Van Broeckhoven C., Collen D., Andersen P.M., Carmeliet P. VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat. Genet., 34: 383-394, 2003. (Pubitemid 36935331)
80. Lariviere R.C. and Julien J.P. Functions of intermediate filaments in neuronal development and disease. J. Neurobiol., 58: 131-148, 2004. (Pubitemid 37543344)
81. Lau D., Ogbogu U., Taylor B., Stafinski T., Menon D., Caulfield T. Stem cell clinics online: the directto-consumer portrayal of stem cell medicine. Cell Stem Cell, 3: 591-594, 2008.
82. Lefaucheur J.P. Principles of therapeutic use of transcranial and epidural cortical stimulation. Clin. Neurophysiol., 119: 2179-2184, 2008
83. Lenaz G., Bovina C., D'Aurelio M., Fato R., Formiggini G., Genova M.L., Giuliano G., Merlo Pich M., Paolucci U., Parenti Castelli G., Ventura B. Role of mitochondria in oxidative stress and ageing. Ann. N.Y. Acad. Sci., 959: 199-213, 2002. (Pubitemid 34457563)
84. Leng Y., Liang M.H., Ren M., Marinova Z., Leeds P., Chuang D.M. Synergistic neuroprotective effects of lithium and valproic acid or other histone deacetylase inhibitors in neurons: roles of glycogen synthase kinase-3 inhibition. J. Neurosci., 28: 2576-2588, 2008. (Pubitemid 351348211)
85. Longstreth W.T., Nelson L.M., Koepsell T.D., van Belle G. Hypotheses to explain the association between vigorous physical activity and amyotrophic lateral sclerosis. Med. Hypotheses, 34: 144-148, 1991.
86. Mackenzie I.R., Rademakers R., Neumann M. TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Lancet Neurol., 9: 995-1007, 2010.
87. Madeo F., Eisenberg T., Kroemer G. Autophagy for the avoidance of neurodegeneration. Genes. Dev., 23: 2253-2259, 2009.
88. Manfredi G. and Xu Z. Mitochondrial dysfunction and its role in motor neuron degeneration in ALS. Mitochondrion, 5: 77-87, 2005. (Pubitemid 40485768)
89. Matthews R.T., Yang L., Browne S., Baik M., Beal M.F. Coenzyme Q10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects. Proc. Natl. Acad. Sci. U.S.A., 95: 8892-8897, 1998.
90. McGeer P.L. and McGeer E.G. Inflammatory processes in amyotrophic lateral sclerosis. Muscle Nerve, 26: 459-470, 2002.
91. Milner-Brown H.S. and Miller R.G. Muscle strengthening through highresistance weight training in patients with neuromuscular disorders. Arch. Phys. Med. Rehabil., 69: 14-19, 1988. (Pubitemid 18040361)
92. Morland C., Boldingh K.A., Iversen E.G., Hassel B. Valproate is neuroprotective against malonate toxicity in rat striatum: an association with augmentation of high-affinity glutamate uptake. J. Cereb. Blood Flow Metab., 24: 1226-1234, 2004. (Pubitemid 39482484)
93. Nau R., Prange H.W., Muth P., Mahr G., Menck S., Kolenda H., Sörgel F. Passage of cefotaxime and ceftriaxone into cerebrospinal fluid of patients with uninflamed meninges. Antimicrob. Agents Chemother., 37: 1518-1524: 1993. (Pubitemid 23202383)
94. Norris F.H. Ceftriaxone in amyotrophic lateral sclerosis. Arch. Neurol., 51: 447, 1994.
95. Okouchi M., Ekshyyan O., Maracine M., Aw T.Y. Neuronal apoptosis in neurodegeneration. Antioxid. Redox Signal., 9: 1059-1096, 2007. (Pubitemid 47080820)
96. Pagani M.R., Reisin R.C., Uchitel O.D. Calcium signaling pathways mediating synaptic potentiation triggered by amyotrophic lateral sclerosis IgG in motor nerve terminals. J. Neurosci., 26: 2661-2672, 2006. (Pubitemid 43357849)
97. Pasquali L., Longone P., Isidoro C., Ruggieri S., Paparelli A., Fornai F. Autophagy, lithium, and amyotrophic lateral sclerosis. Muscle Nerve, 40: 173-194, 2009.
98. Patel M. and Day B.J. Metalloporphyrin class of therapeutic catalytic antioxidants. Trends Pharmacol. Sci., 20: 359-364, 1999. (Pubitemid 29421616)
99. Paulus W., Classen J., Cohen L.G., Large C.H., Lazzaro V.D., Nitsche M., Pascual-Leone A., Rosenow F., Rothwell J.C., Ziemann U. State of the art: pharmacologic effects on cortical excitability measures tested by transcranial magnetic stimulation. Brain Stimulation, 1: 151-163, 2008.
100. Pizzasegola C., Caron I., Daleno C., Ronchi A., Minoia C., Carri M.T., Bendotti C. Treatment with lithium carbonate does not improve disease progression in two different strains of SOD1 mutant mice. Amyotroph. Lateral. Scler., 10: 221-228, 2009.
101. Plaitakis A. and Caroscio J.T. Abnormal glutamate metabolism in amyotrophic lateral sclerosis. Ann. Neurol., 22: 575-579, 1987. (Pubitemid 17162898)
102. Portegies P. and Cohen E.S. Possible etiological role retroviruses and enteroviruses in the development of amyotrophic lateral sclerosis. Ned Tijdschr Geneeskd, 146: 1398-1400, 2002. (Pubitemid 34815063)
103. Przedborski S. Programmed cell death in amyotrophic lateral sclerosis: a mechanism of pathogenic and therapeutic importance. Neurologist, 10: 1-7, 2004. (Pubitemid 38140834)
104. Qureshi M.M., Hayden D., Urbinelli L., Ferrante K., Newhall K., Myers D., Hilgenberg S., Smart R., Brown R.H., Cudkowicz M.E. Analysis of factors that modify susceptibility and rate of progression in amyotrophic lateral sclerosis (ALS). Amyotroph. Lateral Scler., 7: 173-182, 2006. (Pubitemid 44644572)
105. Ravikumar B., Vacher C., Berger Z., Davies J.E., Luo S., Oroz L.G., Scaravilli F., Easton D.F., Duden R., O'Kane C.J., Rubinsztein D.C. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat. Genet., 36: 585-595, 2004. (Pubitemid 38715985)
106. Ridding M.C. and Rothwell J.C. Is there a future for therapeutic use of transcranial magnetic stimulation? Nat. Rev., 8: 559-567, 2007 (Pubitemid 46985363)
107. Robberecht W. Lack of improvement with ceftriaxone in motoneuron disease. Lancet, 340: 1096-1097, 1992.
108. Rosen D.R. Mutations in Cu/Zn superoxide dismutase gene are associated with amyotrophic lateral sclerosis. Nature, 362: 59-62, 1993. (Pubitemid 23087289)
109. Rothstein J.D., Martin L.J., Kuncl R.W. Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. N. Engl. J. Med., 326: 1464-1468, 1992.
110. Rothstein J.D., Jin L., Dykes-Hoberg M., Kuncl R.W. Chronic inhibition of glutamate uptake produces a model of slow neurotoxicity. Proc. Natl. Acad. Sci. U.S.A., 90: 6591-6595, 1993. (Pubitemid 23207259)
111. Rothstein J.D., Van Kammen M., Levey A.I., Martin L.J., Kuncl R.W. Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis. Ann. Neurol., 38: 73-84, 1995.
112. Rothstein J.D. Excitotoxicity hypothesis. Neurology, 47 (Suppl. 2): S19-S25, 1996.
113. Rothstein J.D., Patel S., Regan M.R., Haenggeli C., Huang Y.H., Bergles D.E., Jin L., Dykes Hoberg M., Vidensky S., Chung D.S., Toan S.V., Bruijn L.I., Su Z.Z., Gupta P., Fisher P.B. Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression. Nature, 433: 73-77, 2005. (Pubitemid 40101145)
114. Rouaux C., Panteleeva I., Rene F., Gonzalez de Aguilar J.L., Echaniz-Laguna A., Dupuis L., Menger Y., Boutillier A.L., Loeffler J.P. Sodium valproate exerts neuroprotective effects in vivo through CREB-binding proteindependent mechanisms but does not improve survival in an amyotrophic lateral sclerosis mouse model. J. Neurosci., 27: 5535-5545, 2007. (Pubitemid 350021087)
115. Rubinsztein D.C., Gestwicki J.E., Murphy L.O., Klionsky D.J. Potential therapeutic applications of autophagy. Nat. Rev. Drug. Discov., 6: 304-312, 2007. (Pubitemid 46505881)
116. Sarbassov D.D., Ali S.M., Sabatini D.M. Growing roles for the mTOR pathway. Curr. Opin. Cell. Biol., 17: 596-603, 2005. (Pubitemid 41540408)
117. Sarkar S., Floto R.A., Berger Z., Imarisio S., Cordenier A., Pasco M., Cook LJ, Rubinsztein, D.C. Lithium induces autophagy by inhibiting inositol monophosphatase. J. Cell. Biol., 170: 1101-1111, 2005. (Pubitemid 41362642)
118. Sarkar S., Krishna G., Imarisio S., Saiki S., O'Kane C.J., Rubinsztein D.C. A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin. Hum. Mol. Genet., 17: 170-178, 2008.
119. Senger D.R., Galli S.J., Dvorak A.M., Perruzzi C.A., Harvey V.S., Dvorak H.F. Tumor cells secrete a vascular permeability factor that promotes accumulation of ascites fluid. Science, 219: 983-985, 1983.
120. Shatunov A., Mok K., Newhouse S., Weale M.E., Smith B., Vance C., Johnson L., Veldink J.H., van Es M.A., van den Berg L.H., Robberecht W., Van Damme P., Hardiman O., Farmer A.E., Lewis C.M., Butler A.W., Abel O., Andersen P.M., Fogh I., Silani V., Chiò A., Traynor B.J., Melki J., Meininger V., Landers J.E., McGuffin P., Glass J.D., Pall H., Leigh P.N., Hardy J., Brown R.H. Jr, Powell J.F., Orrell R.W., Morrison K.E., Shaw P.J., Shaw C.E., Al-Chalabi A. Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. Lancet Neurol., 2010 in press.
121. Shaw P.J., Forrest V., Ince P.G., Richardson J.P., Wastell H.J. SF and plasma amino acid levels in motor neuron disease: elevation of CSF glutamate in a subset of patients. Neurodegeneration, 4: 209-216, 1995a.
122. Shaw P., Ince P., Falkous G., Mantel D. Oxidative damage to protein in sporadic motor neuron disease spinal cord. Ann. Neurol., 38: 691-695, 1995b.
123. Shibata N., Nagai R., Uchida K., Horiuchi S., Yamada S., Hirano A., Kawaguchi M., Yamamoto T., Sasaki S., Kobayashi M. Morphological evidence for lipid peroxidation and protein glycoxidation in spinal cords from sporadic amyotrophic lateral sclerosis patients. Brain Res., 917: 97-104, 2001. (Pubitemid 32972133)
124. Shichinohe H., Kuroda S., Yasuda H., Ishikawa T., Iwai M., Horiuchi M., Iwasaki Y. Neuroprotective effects of the free radical scavenger Edaravone (MCI-186) in micepermanent focal brain ischemia. Brain Res., 1029: 200-206, 2004. (Pubitemid 39470176)
125. Shin J.H., Cho S.I., Lim H.R., Lee J.K., Lee Y.A., Noh J.S., Joo I.S., Kim K.W., Gwag B.J. Concurrent administration of Neu2000 and lithium produces marked improvement of motor neuron survival, motor function, and mortality in a mouse model of amyotrophic lateral sclerosis. Mol. Pharmacol., 71: 965-975, 2007.
126. Siciliano G., D'Avino C., Del Corona A., Barsacchi R., Kusmic C., Rocchi A., Pastorini E., Murri L. Impaired oxidative metabolism and lipid peroxidation in exercising muscle from ALS patients. Amyotroph. Lateral Scler. Other Motor Neuron Disord., 3: 57-62, 2002.
127. Siciliano G., Piazza S., Carlesi C., Del Corona A., Franzini M., Pompella A., Malvaldi G., Mancuso M., Paolicchi A., Murri L. Antioxidant capacity and protein oxidation in cerebrospinal fluid of amyotrophic lateral sclerosis. J. Neurol., 254: 575-580, 2007. (Pubitemid 46781962)
128. Siciliano G., Carlesi C., Pasquali L., Piazza S., Pietracupa S., Fornai F., Ruggieri S., Murri L. Clinical trials for neuroprotection in ALS. CNS Neurol. Disord. Drug Targets, 9: 305-313, 2010.
129. Silani V., Cova L., Corbo M., Ciammola A., Polli E. Stem-cell therapy for amyotrophic lateral sclerosis. Lancet, 364: 200-202, 2004. (Pubitemid 38891692)
130. Simpson C.L. and Al-Chalabi A. Amyotrophic lateral sclerosis as a complex genetic disease. Biochim. Biophys. Acta, 1762: 973-985, 2006. (Pubitemid 44768086)
131. Smith R.G., Henry Y.K., Mattson M.P., Appel S.H. Presence of 4-hydroxynonenal in cerebrospinal fluid of patients with sporadic amyotrophic lateral sclerosis. Ann. Neurol., 44: 696-699, 1998. (Pubitemid 28477861)
132. Smith R.A., Miller T.M., Yamanaka K., Monia B.P., Condon T.P., Hung G., Lobsiger C.S., Ward C.M., McAlonis-Downes M., Wei H., Wancewicz E.V., Bennett C.F., Cleveland D.W. Antisense oligonucleotide therapy for neurodegenerative disease. J. Clin. Invest., 116: 2290-2296, 2006. (Pubitemid 44162339)
133. Sondell M., Sundler F., Kanje M. Vascular endothelial growth factor is a neurotrophic factor which stimulates axonal outgrowth through the flk-1 receptor. Eur. J. Neurosci., 12: 4243-4254, 2000. (Pubitemid 32037835)
134. Sorenson E.J., Windbank A.J., Mandrekar J.N., Bamlet W.R., Appel S.H., Armon C., Barkhaus P.E., Bosch P., Boylan K., David W.S., Feldman E., Glass J., Gutmann L., Katz J., King W., Luciano C.A., McCluskey L.F., Nash S., Newman D.S., Pascuzzi R.M., Pioro E., Sams L.J., Scelsa S., Simpson E.P., Subramony S.H., Tiryaki E., Thornton C.A. Subcutaneous IGF-1 is not beneficial in 2-year ALS trial. Neurology, 71: 1770-1775, 2008.
135. Stambolic V., Ruel L., Woodgett J.R. Lithium inhibits glycogen synthase kinase-3 activity and mimics wingless signaling in intact cells. Curr. Biol., 6: 1664-1668, 1996. (Pubitemid 27050234)
136. Stommel E.W., Cohen J.A., Fadul C.E., Cogbill C.H., Graber D.J., Kingman L., Mackenzie T., Channon Smith J.Y., Harris B.T. Efficacy of thalidomide for the treatment of amyotrophic lateral sclerosis: A phase II open label clinical trial. Amyotroph. Lateral Scler., 10: 393-404, 2009.
137. Sugai F., Yamamoto Y., Miyaguchi K., Zhou Z., Sumi H., Hamasaki T., Goto M., Sakoda S. Benefit of valproic acid in suppressing disease progression of ALS model mice. Eur. J. Neurosci., 20: 3179-3183, 2004. (Pubitemid 40007312)
138. Traynor B.J., Bruijn L., Conwit R., Beal F., O'Neill G., Fagan S.C., Cudkowicz M.E. Neuroprotective agents for clinical trials in ALS: a systematic assessment. Neurology, 67: 20-27, 2006. (Pubitemid 44305456)
139. Uno M., Kitazato K.T., Suzue A., Matsuzaki K., Harada M., Itabe H., Nagahiro S. Inhibition of brain damage by edaravone, a free radical scavenger,
140. Van Den Bosch L., Van Damme P., Bogaert E., Robberecht W. The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis. Biochim. Biophys. Acta, 1762: 1068-1082, 2006. (Pubitemid 44781176)
141. Van Den Bosch L., Tillkin P., Lemmens G., Robberecht W. Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport, 13: 1067-1070, 2002. (Pubitemid 34587742)
142. Veldink J.H., Kalmijn S., Groeneveld G.J., Titulaer M.J., Wokke J.H., van den Berg L.H. Physical activity and the association with sporadic ALS. Neurology, 64: 241-245, 2005.
143. Venkatachalam K., Long A.A., Elsaesser R., Nikolaeva D., Broadie K., Montell C. Motor deficit in a Drosophila model of mucolipidosis type IV due to defective clearance of apoptotic cells. Cell, 135: 838-851, 2008.
144. Wang Y., Mao X.O., Xie L., Banwait S., Marti H. H., Greenberg D.A., Jin K. Vascular endothelial growth factor overexpression delays neurodegen-eration and prolongs survival in amyotrophic lateral sclerosis mice. J. Neurosci., 27: 304-307, 2007. (Pubitemid 46106003)
145. Watanabe T., Yuki S., Egawa M., Nishi H. Protective effects of MCI-186 on cerebral ischemia: possible involvement of free radical scavenging and antioxidant actions. J. Pharmacol. Exp. Ther., 268: 1597-1604, 1994. (Pubitemid 24100383)
146. Westerheide S.D., Bosman J.D., Mbadugha B.N., Kawahara T.L., Matsumoto G., Kim S., Gu W., Devlin J.P., Silverman R.B., Morimoto R.I. Celastrols as inducers of the heat shock response and cytoprotection. J. Biol. Chem., 279: 56053-56060, 2004. (Pubitemid 40066613)
147. Wicklund M.P. Amyotrophic lateral sclerosis: possible role of environmental influences. Neurol. Clin., 23: 461-484, 2005. (Pubitemid 40332610)
148. Wood J.D., Beaujeux T.P., Shaw P.J. Protein aggregation in motor neurone disorders. Neuropathol. Appl. Neurobiol., 29: 529-545, 2003. (Pubitemid 37548348)
149. Wu D.C., Re D.B., Nagai M., Ischiropoulos H., Przedborski S. The inflammatory NADPH oxidase enzyme modulates motor neuron degeneration in amyotrophic lateral sclerosis mice. Proc. Natl. Acad. Sci. U. S. A., 103: 12132-12137, 2006. (Pubitemid 44215823)
150. Xu J.X. Radical metabolism is partner to energy metabolism in mitochondria. Ann. N.Y. Acad. Sci., 1011: 57-60, 2004.
151. Yang Q. and Guan K.L. Expanding mTOR signaling. Cell Res., 17: 666-681, 2007. (Pubitemid 47255937)
152. Yoshino H. and Kimura A. Investigation of the therapeutic effects of edaravone, a free radical scavenger, on amyotrophic lateral sclerosis (Phase II study). Amyotroph. Lateral. Scler., 7: 241-245, 2006.
153. Zheng C., Nennesmo I., Fadeel B., Henter J.I. Vascular endothelial growth factor prolongs survival in a transgenic mouse model of ALS. Ann. Neurol., 56: 564-567, 2004. (Pubitemid 39319348)