Transgenic models of amyotrophic lateral sclerosis

Folia Neuropathologica

Volumn 42, Issue 4, 2004, Pages 239-248

  Grieb, Paweł ^a  

^a MOSSAKOWSKI MEDICAL RESEARCH CENTRE   (Poland)

Author keywords

Amyotrophic lateral sclerosis; Transgenic disease models

Indexed keywords

4 (4 HYDROXYANILINO) 6,7 DIMETHOXYQUINAZOLINE; ACETIC ACID DERIVATIVE; ACETYLCYSTEINE; ALANINE; ALPHA METHYLGUANIDINOACETIC ACID; COPPER ZINC SUPEROXIDE DISMUTASE; CYCLOOXYGENASE 2 INHIBITOR; GABAPENTIN; GLATIRAMER; GLUTAMIC ACID; JANUS KINASE 3 INHIBITOR; LYSINE; MANGANESE DERIVATIVE; MANGANESE N,N' ETHYLENEBIS(3 METHOXYSALICYLIDENEAMINE); NIMESULIDE; RILUZOLE; SOMATOMEDIN C; TETRACYCLINE; TYROSINE; UNCLASSIFIED DRUG; VINCRISTINE;

AMYOTROPHIC LATERAL SCLEROSIS; CATALYSIS; DISEASE MODEL; DRUG MECHANISM; FAMILIAL DISEASE; FATALITY; FREQUENCY ANALYSIS; GENE EXPRESSION; GENE MUTATION; GENE SILENCING; GENE THERAPY; HUMAN; HUMAN VERSUS ANIMAL COMPARISON; HYPOTHESIS; MOTONEURON; NERVE CELL DEGENERATION; NEUROPATHOLOGY; NONHUMAN; OLIGOMERIZATION; PARALYSIS; PHENOTYPE; REVIEW; RODENT; STEM CELL TRANSPLANTATION; TRANSGENICS; VIRAL GENE DELIVERY SYSTEM; VIRAL GENE THERAPY;

EID: 12344293521     PISSN: 16414640     EISSN: 1509572X     Source Type: Journal    
DOI: None     Document Type: Review

References (101)

1. Andreassen OA, Jenkins BG, Dedeoglu A, Ferrante KL, Bogdanov MB, Kaddurah-Daouk R, Beal MF (2001) Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation. J Neurochem, 77: 383-390.
2. Angelov DN, Waibel S, Guntinas-Lichius O, Lenzen M, Neiss WF, Tomov TL, Yoles E, Kipnis J, Schori H, Reuter A, Ludolph A, Schwartz M (2003) Therapeutic vaccine for acute and chronic motor neuron diseases: implications for amyotrophic lateral sclerosis. Proc Natl Acad Sci USA, 100: 4790-4795.
3. Bajaj NP, Al-Sarraj ST, Anderson V, Kibble M, Leigh N, Miller CC (1998) Cyclin-dependent kinase-5 is associated with lipofuscin in motor neurones in amyotrophic lateral sclerosis. Neurosci Lett, 245: 45-48.
4. Bartnikas TB, Gitlin JD (2003) Mechanisms of biosynthesis of mammalian copper/zinc superoxide dismutase. J Biol Chem, 278: 33602-33608.
5. Batulan Z, Shinder GA, Minotti S, He BP, Doroudchi MM, Nalbantoglu J, Strong MJ, Durham HD (2003) High threshold for induction of the stress response in motor neurons is associated with failure to activate HSF1. J Neurosci, 23: 5789-5798.
6. Bendotti C, Atzori C, Piva R, Tortarolo M, Strong MJ, DeBiasi S, Migheli A (2004) Activated p38MAPK is a novel component of the intracellular inclusions found in human amyotrophic lateral sclerosis and mutant SOD1 transgenic mice. J Neuropathol Exp Neurol, 63: 113-119.
7. Bruce KM, Narayan K, Kong HC, Larmour I, Lopes EC, Turner BJ, Bertram JF, Cheema SS (2004) Chemotherapy delays progression of motor neuron disease in the SOD1 G93A transgenic mouse. Chemotherapy, 50: 138-142.
8. Burns C, Wilks A, Su S (2004) JAK kinase inhibitors. Frug Discov Today 9: 694-695.
9. Chung YH, Joo KM, Lee YJ, Lee WB, Lee KH, Cha CI (2004) Enhanced expression of erythropoietin in the central nervous system of SOD1(G93A)transgenic mice. Brain Res, 1016:272-280.
10. Cleveland DW, Liu J (2000) Oxidation versus aggregation: howdo SOD-1 mutants cause ALS? Nat Med, 6: 1320-1321.
11. Cleveland DW, Rothstein JD (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci, 2: 806-819.
12. Consilvio C, Vincent AM, Feldman EL (2004) Neuroinflammation, COX-2, and ALS - a dual role? Exp Neurol, 187: 1-10.
13. Couillard-Despres S, Zhu Q, Wong PC, Price DL, Cleveland DW, Julien JP (1998) Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase. Proc Natl Acad Sci USA, 95:9626-9630.
14. Cutler RG, Pedersen WA, Camandola S, Rothstein JD, Mattson MP (2002) Evidence that accumulation of ceramides and cholesterol esters mediates oxidative stress-induced death of motor neurons in amyotrophic lateral sclerosis. Ann Neurol, 52: 448-457.
15. Derave W, Van Den Bosch L, Lemmens G, Eijnde BO, Robberecht W, Hespel P (2003) Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment. Neurobiol Dis, 13: 264-272.
16. Desport JC, Preux PM, Magy L, Boirie Y, Vallat JM, Beaufrere B, Couratier P (2001) Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis. Am J Clin Nutr, 74: 328-334.
17. Dib M (2003) Amyotrophic lateral sclerosis: progress and prospects for treatment. Drugs, 63: 289-310.
18. Diguet E, Gross CE, Tison F, Bezard E (2004) Rise and fall of minocycline in neuroprotection: need to promote publication of negative results. Exp Neurol, 189: 1-4.
19. Drory VE, Gross D (2002) No effect of creatine on respiratory distress in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord, 3: 43-46.
20. Dupuis L, di Scala F, Rene F, de Tapia M, Oudart H, Pradat PF, Meininger V, Loeffler JP (2003) Up-regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis. FASEB J, 17: 2091-2093.
21. Gao B, Flores SC, Leff JA, Bose SK, McCord JM (2003) Synthesis and anti-inflammatory activity of a chimeric recombinant superoxide dismutase: SOD2/3. Am J Physiol Lung Cell Mol Physiol, 284: L917-L925.
22. Garbuzova-Davis S, Willing AE, Zigova T, Saporta S, Justen EB, Lane JC, Hudson JE, Chen N, Davis CD, Sanberg PR (2003) Intravenous administration of human umbilical cord blood cells in a mouse model of amyotrophic lateral sclerosis: distribution, migration, and differentiation. J Hematother Stem Cell Res, 12: 255-270.
23. Gong YH, Parsadanian AS, Andreeva A, Snider WD, Elliott JL (2000) Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration. J Neurosci, 20: 660-665.
24. Groeneveld GJ, de Leeuw van Weenen J, van Muiswinkel FL, Veldman H, Veldink JH, Wokke JH, Bar PR, van den Berg LH (2003b) Zinc amplifies mSOD1-mediated toxicity in a transgenic mouse model of amyotrophic lateral sclerosis. Neurosci Lett, 352: 175-178.
25. Groeneveld GJ, Veldink JH, van der Tweel I, Kalmijn S, Beijer C, de Visser M, Wokke JH, Franssen H, van den Berg LH (2003a) A randomized sequential trial of creatine in amyotrophic lateral sclerosis. Ann Neurol, 53: 437-445.
26. Guegan C, Przedborski S (2003) Programmed cell death in amyotrophic lateral sclerosis. J Clin Invest, 111: 153-161.
27. Gurney ME (1997) The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies. J Neurol Sci, 152 (Suppl 1): S67-S73.
28. Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng HX, Chen W, Zhai P, Sufit RL, Siddique T (1994) Motor neuron degeneration in mice that express a human Cu/Zn superoxide gene mutation. Science, 264: 1772-1775.
29. Hafezparast M, Ahmad-Annuar A, Hummerich H, Shah P, Ford M, Baker C, Bowen S, Martin JE, Fisher EM (2003) Paradigms for the identification of new genes in motor neuron degeneration. Amyotroph Lateral Scler Other Motor Neuron Disord, 4:249-257.
30. Haverkamp LJ, Appel V, Appel SH (1995) Natural history of amyotrophic lateral sclerosis in a database population: validation of a scoring system and a model for survival prediction. Brain, 118: 707-719.
31. Hayward LJ, Rodriguez JA, Kim JW, Tiwari A, Goto JJ, Cabelli DE, Valentine JS, Brown RH Jr (2002) Decreased metallation and activity in subset of mutant superoxide dismutase associated with familial amyotrophic lateral sclerosis. J Biol Chem, 277: 15923-15931.
32. Heath PR, Shaw PJ (2002) Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophic lateral sclerosis. Muscle Nerve, 26: 438-458.
33. Hensley K, Fedynyshyn J, Ferrel S, Floyd RA, Gordon G, Grammas P, Hamdheydari L, Mhatre M, Mou S, Pye QN, Stewart C, West M, West S, Williamson KS (2003) Message and protein-level elevation of tumor necrosis factor alpha (TNF alpha) and TNF alpha-modulating cytokines in spinal cords of the G93A-SOD1 mouse model for amyotrophic lateral sclerosis. Neurobiol Dis, 14: 74-80.
34. Higgins CM, Jung C, Xu Z (2003) ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes. BMC Neurosci, 4: 16.
35. Hinerfeld D, Traini MD, Weinberger RP, Cochran B, Doctrow SR, Harry J, Melov S (2004) Endogenous mitochondrial oxidative stress: neurodegeneration, proteomic analysis, specific respiratory chain defects, and efficacious antioxidant therapy in superoxide dismutase 2 null mice. J Neurochem, 88: 657-667.
36. Howland DS, Liu J, She Y, Goad B, Maragakis NJ, Kim B, Erickson J, Kulik J, DeVito L, Psaltis G, DeGennaro LJ, Cleveland DW, Rothstein JD (2002) Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci USA, 99: 1604-1609.
37. Hu JH, Chernoff K, Pelech S, Krieger C (2003b) Protein kinase and protein phosphatase expression in the central nervous system of G93A mSOD over-expressing mice. J Neurochem, 85: 422-431.
38. Hu JH, Zhang H, Wagey R, Krieger C, Pelech SL (2003a) Protein kinase and protein phosphatase activation in amyotrophic lateral sclerosis spinal cord. J Neurochem, 85: 432-442.
39. Inoue H, Tsukita K, Iwasato T, Suzuki Y, Tomioka M, Tateno M, Nagao M, Kawata A, Saido TC, Miura M, Misawa H, Itohara S, Takahashi R (2003a) The crucial role of caspase-9 in the disease progression of atransgenic ALS mouse model. EMBO J, 22: 6665-6674.
40. Inoue M, Sato EF, Nishikawa M, Park AM, Kira Y, Imada I, Utsumi K (2003b) Cross talk of nitric oxide, ozygen radicals, and superoxide dismutase regulates the energy metabolism and cell death and determines the fates of aerobic life. Antioxid Redox Signal, 5: 475-484.
41. Jaarsma D, Rognoni F, van Duijn W, Verspaget HW, Haasdijk ED, Holstege JC (2001) CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked mutations. Acta Neuropathol, 102: 293-305.
42. Johnston JA, Illing ME, Kopito RR (2002) Cytoplasmic dynein/dynactin mediates the assembly of aggresomes. Cell Motil Cytoskeleton, 53: 26-38.
43. Julien J-P (2001) Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded. Cell, 104: 581-591.
44. Kaspar BK, Llado J, Sherkat N, Rothstein JD, Gage FH (2003) Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model. Science, 301: 839-842.
45. Kato S, Saeki T, Aoki M, Nagai M, Ishigaki A, Itoyama Y, Kato M, Asayama K, Awaya A, Hirano A, Ohama E (2003) Histological evidence of redox system breakdown caused by superoxide dismutase 1 (SOD1) aggregation is common to SOD1-mutated motor neurons in humans and animal models. Acta Neuropathol, 112-120.
46. Kieran D, Kalmar B, Dick JR, Riddoch-Contreras J, Burnstock G, Greensmith L (2004) Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat Med, 10: 402-405.
47. Klivenyi P, Ferrante RJ, Matthews RT, Bogdanov MB, Klein AM, Andreassen OA, Mueller G, Wermer M, Kaddurah-Daouk R, Beal MF (1999) Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis. Nat Med, 5: 347-350.
48. Klivenyi P, Kiaei M, Gardian G, Calingasan NY, Beal MF (2004) Additive neuroprotective effects of creatine and cyclooxygenase 2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurochem, 88: 576-582.
49. Kong J, Xu Z (1998) Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J Neurosci, 18: 3241-3250.
50. Kong J, Xu Z (2000) Overexpression of neurofilament sub-unit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis. Neurosci Lett, 281: 72-74.
51. Kriz J, Gowing G, Julien JP (2003) Efficient three-drug cocktail for disease induced by mutant superoxide dismutase. Ann Neurol, 53: 429-436.
52. Kriz J, Nguyen MD, Julien JP (2002) Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol Dis, 10: 268-278.
53. Lebovitz RM, Zhang H, Vogel H, Cartwright J Jr, Dionne L, Lu N, Huang S, Matzuk MM (1996) Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice. Proc Natl Acad Sci USA, 93: 9782-9787.
54. Lindberg MJ, Tibell L, Oliveberg M (2002) Common denominator of Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis: Decreased stability of the apo state. Proc Natl Acad Sci USA, 99: 16607-16612.
55. Lino MM, Schneider C, Caroni P (2002) Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease. J Neurosci, 22: 4825-4832.
56. Liochev SI, Fridovich I (1999) Superoxide and iron: partners in crime. IUBMB Life, 48: 157-161.
57. Liochev SI, Fridovich I (2003) Mutant Cu,Zn superoxide dismutases and familial amyotrophic lateral sclerosis: evaluation of oxidative hypotheses. Free Radic Biol Med, 34: 1383-1389.
58. Liochev SI, Fridovich I (2004) CO2, not HCO3-, facilitates oxidations by Cu,Zn superoxide dismutase plus H2O2. Proc Natl Acad Sci USA, 101: 743-744.
59. Liu J, Lillo C, Jonsson PA, Velde CV, Ward CM, Miller TM, Subramaniam JR, Rothstein JD, Marklund S, Andersen PM, Brannstrom T, Gredal O, Wong PC, Williams DS, Cleveland DW (2004) Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron, 43: 5-17.
60. Maatkamp A, Vlug A, Haasdijk E, Troost D, French PJ, Jaarsma D (2004) Decrease of Hsp25 protein expression precedes degeneration of motoneurons in ALS-SOD1 mice. Eur J Neurosci, 20: 14-28.
61. Mattiazzi M, D'Aurelio M, Gajewski CD, Martushova K, Kiaei M, Beal MF, Manfredi G (2002) Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem, 277: 29626-29633.
62. McGeer PL, McGeer EG (2002) Inflammatory processes in amyotrophic lateral sclerosis. Muscle Nerve, 26: 459-470.
63. Miller RG, Mitchell JD, Lyon M, Moore DH (2002) Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev CD001447.
64. Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, Brown RH Jr, Itoyama Y (2001) Rats overexpressing human cytosolic copper-zinc superoxide dismutase transgenes with apyotrophic lateral sclerosis-associated mutations develop motor neuron disease. J Neurosci, 21: 9246-9254.
65. Nguyen MD, Boudreau M, Kriz J, Couillard-Despres, Kaplan DH, Julien J-P (2003) Cell cycle regulators in the neuronal death pathway of amyotrophic lateral sclerosis caused by mutant superoxide dismutase 1. J Neurosci, 23:2131-2140.
66. Nguyen MD, Lariviere RC, Julien JP (2001) Deregulation of Cdk5 in a mouse model of ALS: toxicity alleviated by perikaryal neurofilament inclusions. Neuron, 30: 135-147.
67. Okado-Matsumoto A, Fridovich I (2002) Amyotrophic lateral sclerosis: A proposed mechanism. Proc Natl Acad Sci USA, 99: 9010-9014.
68. Pasinelli P, Belford ME, Lennon N, Bacskai BJ, Hyman BT, Trotti D, Brown RH Jr (2004) Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron, 43: 19-30.
69. Pompl PN, Ho L, Bianchi M, McManus T, Qin W, Pasinetti GM (2003) A therapeutic role for cyclooxygenase-2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis. FASEB J, 17: 725-727.
70. Pramatarova A, Laganiere J, Roussel J, Brisebois K, Rouleau GA (2001) Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment. J Neurosci, 21: 3369-3374.
71. Przedborski S, Mitsumoto H, Rowland LP (2003) Recent advances in amyotrophic lateral sclerosis research. Curr Neurol Neurosci Rep, 3: 70-77.
72. Raiteri L, Stigliani S, Zappettini S, Mercuri NB, Raiteri M, Bonanno G (2004) Excessive and precocious glutamate release in a mouse model of amyotrophic lateral sclerosis. Neuropharmacology, 46: 782-792.
73. Rakhit R, Cunningham P, Furtos-Matei A, Dahan S, Qi XF, Crow JP, Cashman NR, Kondejewski LH, Chakrabartty A (2002) Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis. J Biol Chem, 277: 47551-47556.
74. Reaume AG, Elliott JL, Hoffman EK, Kowall NW, Ferrante RJ, Siwek DF, Wilcox HM, Flood DG, Beal MF, Brown RH Jr, Scott RW, Snider WD (1966) Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat Genet, 13: 43-47.
75. Rembach A, Turner BJ, Bruce S, Cheah IK, Scott RL, Lopes EC, Zagami CJ, Beart PM, Cheung NS, Langford SJ, Cheema SS (2004) Antisense peptide nucleic acid targeting GluR3 delays disease onset and progression in the SOD1 G93A mouse model of familial ALS. J Neurosci Res, 77: 573-582.
76. Ripps ME, Huntley GW, Hof PR, Morrison JH, Gordon GW (1995) Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA, 92: 689-693.
77. Robertson J, Kriz J, Nguyen MD, Nguyen MD, Julien J-P (2002) Pathways to motor neuron degeneration in transgenic mouse models. Biochimie, 84: 1151-1160.
78. Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O'Regan JP, Deng HX, et al. (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature, 362: 59-62.
79. Rowland LP, Shneider NA (2001) Amyotrophic lateral sclerosis. N Engl J Med, 344: 1688-1700.
80. Sasaki S, Ohsawa Y, Yamane K, Sakuma H, Shibata N, Nakano R, Kikugawa K, Mizutani T, Tsuji S, Iwata M (1988) Familial amyotrophic lateral sclerosis with widespread vacuolation and hyaline inclusions. Neurology, 51: 871-873.
81. Schrauwen P, Hesselink MK (2004) The role of uncoupling protein 3 in fatty acid metabolism: protection against lipotoxicity? Proc Nutr Soc, 63: 287-292.
82. Shefner JM, Reaume AG, Flood DG, Scott RW, Kowall NW, Ferrante RJ, Siwek OF, Upton-Rice M, Brown RH Jr. (1999) Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy. Neurology, 53: 1239-1246.
83. Shin JH, Yang JW, Le Pecheur M, London J, Hoeger H, Lubec G (2004) Altered expression of hypothetical proteins in hippocampus of transgenic mice overexpressing human Cu/Zn-superoxide dismutase I. Proteome Sci - 2: 2.
84. Silani PV, Cova L, Corbo M, Ciammola A, Prof Polli E (2004) Stem-cell therapy for amyotrophic lateral sclerosis. Lancet 364: 200-202.
85. Simpson EP, Yen AA, Appel SH (2003) Oxidative stress: a common denominator in the pathogenesis of amyotrophic lateral sclerosis. Curr Opin Rheumatol, 15: 730-736.
86. Suzuki YJ, Forman HJ, Sevanian A (1997) Oxidants as stimulators of signal transduction. Free Radic Biol Med, 22: 269-285.
87. Tortarolo M, Veglianese P, Calvaresi N, Botturi A, Rossi C, Giorgini A, Migheli A, Bendotti C (2003) Persistent activation of p38 mitogen-activated protein kinase in a mouse model of familial amyotrophic lateral sclerosis correlates with disease progression. Mol Cell Neurosci, 23: 180-192.
88. Trieu VN, Liu R, Liu XP, Uckun FM (2000) A specific inhibitor of janus kinase-3 increases survival in a transgenic mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun, 267: 22-25.
89. Turner BJ, Lopes EC, Cheema SS (2004) Inducible superoxide dismutase 1 aggregation in transgenic amyotrophic lateral sclerosis mouse fibroblasts. J Cell Biochem, 91: 1074-1084.
90. Turner MR, Parton MJ, Leigh PN (2001) Clinical trials in ALS: an overview. Semin Neurol, 21: 167-175.
91. Valentine JS, Hart PJ (2003) Misfolded CuZnSOD and amyotrophic lateral sclerosis. Proc Natl Acad Sci USA, 100: 3617-3622.
92. Van Den Bosch L, Tilkin P, Lemmens G, Robberecht W (2002) Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport, 13: 1067-1070.
93. Vlug AS, Jaarsma D (2004) Longterm proteasome inhibition does not preferentially afflict motor neurons in organotypical spinal cord cultures. Amyotroph Lateral Scler Other Motor Neuron Disord, 5: 16-21.
94. Williamson TL, Bruijn LI, Zhu Q, Anderson KL, Anderson SD, Julien JP, Cleveland DW (1998) Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant. Proc Natl Acad Sci USA, 95: 9631-9636.
95. Witting A, Weydt P, Hong S, Kliot M, Moller T, Stella N (2004) Endocannabinoids accumulate in spinal cord of SOD1 G93A transgenic mice. J Neurochem, 89: 1555-1557.
96. Wong, PC, Pardo CA, Borchelt DR, Lee MK, Copeland NG, Jenkins NA, Sisodia SS, Cleveland DW, Price DL (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuole degeneration of mitochondria. Neuron, 14: 1105-1116.
97. Wood JD, Beaujeux TP, Shaw PJ (2003) Protein aggregation in motor neurone disorders. Neuropathol Appl Neurobiol, 29: 529-545.
98. Wootz H, Hansson I, Korhonen L, Napankangas U, Lindholm D (2004) Caspase-12 cleavage and increased oxidative stress during neuron degeneration in transgenic mouse model of ALS. Biochem Biophys Res Comm, 322: 281-286.
99. Yoshida S, Mulder DW, Kurland LT, Chu CP, Okazaki H (1984) Follow-up study on amyotrophic lateral sclerosis in Rochester, Minn., 1925 through 1984. Neuroepidemiology, 5: 61-70.
100. Zhang W, Narayanan M, Friedlander RM (2003) Additive neuroprotective effects of minocycline with creatine in a mouse model of ALS. Ann Neurol, 53: 267-270.
101. Zhu S, Stavrovskaya IG, Drozda M, Kim BY, Ona V, Li M, Sarang S, Liu AS, Hartley DM, Wu du C, Gullans S, Ferrante RJ, Przedborski S, Kristal BS, Friedlander RM (2002) Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice. Nature, 417: 74-78.