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Volumn 3, Issue 4, 2006, Pages 331-339

Mouse models of amyotrophic lateral sclerosis

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA TOCOPHEROL; CEFTRIAXONE; COPPER ZINC SUPEROXIDE DISMUTASE; CREATINE; GABAPENTIN; GLIAL CELL LINE DERIVED NEUROTROPHIC FACTOR; LENTIVIRUS VECTOR; LYSINE ACETYLSALICYLATE; MICROTUBULE PROTEIN; MINOCYCLINE; NIMODIPINE; PROTEASOME; PROTEIN ALS2; RECOMBINANT ENZYME; RECOMBINANT VASCULOTROPIN; RILUZOLE; SOMATOMEDIN C; UNCLASSIFIED DRUG;

EID: 33846597946     PISSN: 17406757     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.ddmod.2006.12.003     Document Type: Review
Times cited : (2)

References (76)
  • 1
    • 0027401203 scopus 로고
    • Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    • Rosen D.R., et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362 (1993) 59-62
    • (1993) Nature , vol.362 , pp. 59-62
    • Rosen, D.R.1
  • 2
    • 0034785509 scopus 로고    scopus 로고
    • The gene encoding alsin, a protein with three guaninenucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis
    • Yang Y., et al. The gene encoding alsin, a protein with three guaninenucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat. Genet. 29 (2001) 160-165
    • (2001) Nat. Genet. , vol.29 , pp. 160-165
    • Yang, Y.1
  • 3
    • 0034785483 scopus 로고    scopus 로고
    • A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2
    • Hadano S., et al. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat. Genet. 29 (2001) 166-173
    • (2001) Nat. Genet. , vol.29 , pp. 166-173
    • Hadano, S.1
  • 4
    • 0036724052 scopus 로고    scopus 로고
    • Infantile-onset ascending hereditary spastic paralysis is associated with mutations in the Alsin gene
    • Eymard-Pierre E., et al. Infantile-onset ascending hereditary spastic paralysis is associated with mutations in the Alsin gene. Am. J. Hum. Genet. 71 (2002) 518-527
    • (2002) Am. J. Hum. Genet. , vol.71 , pp. 518-527
    • Eymard-Pierre, E.1
  • 5
    • 0037382240 scopus 로고    scopus 로고
    • Mutant dynactin in motor neuron disease
    • Puls I., et al. Mutant dynactin in motor neuron disease. Nat. Genet. 33 (2003) 455-456
    • (2003) Nat. Genet. , vol.33 , pp. 455-456
    • Puls, I.1
  • 6
    • 2442658908 scopus 로고    scopus 로고
    • DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4)
    • Chen Y.Z., et al. DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4). Am. J. Hum. Genet. 74 (2004) 1128-1135
    • (2004) Am. J. Hum. Genet. , vol.74 , pp. 1128-1135
    • Chen, Y.Z.1
  • 7
    • 6344257200 scopus 로고    scopus 로고
    • A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis
    • Nishimura A.L., et al. A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis. Am. J. Hum. Genet. 75 (2004) 822-831
    • (2004) Am. J. Hum. Genet. , vol.75 , pp. 822-831
    • Nishimura, A.L.1
  • 8
    • 0041903805 scopus 로고    scopus 로고
    • VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death
    • Lambrechts D., et al. VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat. Genet. 34 (2003) 383-394
    • (2003) Nat. Genet. , vol.34 , pp. 383-394
    • Lambrechts, D.1
  • 9
    • 28044435808 scopus 로고    scopus 로고
    • Lack of association between VEGF polymorphisms and ALS in a Dutch population
    • Van Vught P.W., et al. Lack of association between VEGF polymorphisms and ALS in a Dutch population. Neurology 65 (2005) 1643-1645
    • (2005) Neurology , vol.65 , pp. 1643-1645
    • Van Vught, P.W.1
  • 10
    • 8844263662 scopus 로고    scopus 로고
    • A novel candidate region for ALS on chromosome 14q11.2
    • Greenway M.J., et al. A novel candidate region for ALS on chromosome 14q11.2. Neurology 63 (2004) 1936-1938
    • (2004) Neurology , vol.63 , pp. 1936-1938
    • Greenway, M.J.1
  • 11
    • 0021157469 scopus 로고
    • Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis
    • Hirano A., et al. Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis. J. Neuropathol. Exp. Neurol. 43 (1984) 471-480
    • (1984) J. Neuropathol. Exp. Neurol. , vol.43 , pp. 471-480
    • Hirano, A.1
  • 12
    • 0026739393 scopus 로고
    • Peripherin and neurofilament protein coexist in spinal spheroids of motor neuron disease
    • Corbo M., and Hays A.P. Peripherin and neurofilament protein coexist in spinal spheroids of motor neuron disease. J. Neuropathol. Exp. Neurol. 51 (1992) 531-537
    • (1992) J. Neuropathol. Exp. Neurol. , vol.51 , pp. 531-537
    • Corbo, M.1    Hays, A.P.2
  • 13
    • 33747605320 scopus 로고    scopus 로고
    • Molecular biology of amyotrophic lateral sclerosis: insights from genetics
    • Pasinelli P., and Brown R.H. Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat. Rev. Neurosci. 7 (2006) 710-723
    • (2006) Nat. Rev. Neurosci. , vol.7 , pp. 710-723
    • Pasinelli, P.1    Brown, R.H.2
  • 14
    • 15844393658 scopus 로고    scopus 로고
    • Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
    • Reaume A.B., et al. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat. Genet. 13 (1996) 43-47
    • (1996) Nat. Genet. , vol.13 , pp. 43-47
    • Reaume, A.B.1
  • 15
    • 0033231494 scopus 로고    scopus 로고
    • From Charcot to SOD1: mechanisms of selective motor neuron death in ALS
    • Cleveland D.W. From Charcot to SOD1: mechanisms of selective motor neuron death in ALS. Neuron 24 (1999) 515-520
    • (1999) Neuron , vol.24 , pp. 515-520
    • Cleveland, D.W.1
  • 16
    • 0029671220 scopus 로고    scopus 로고
    • Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis
    • Wiedau-Pazos M., et al. Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis. Science 271 (1996) 515-518
    • (1996) Science , vol.271 , pp. 515-518
    • Wiedau-Pazos, M.1
  • 17
    • 0027293275 scopus 로고
    • ALS, SOD and peroxynitrite
    • Beckman J.S., et al. ALS, SOD and peroxynitrite. Nature 364 (1993) 584
    • (1993) Nature , vol.364 , pp. 584
    • Beckman, J.S.1
  • 18
    • 0036212119 scopus 로고    scopus 로고
    • Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading
    • Subramaniam J.R., et al. Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat. Neurosci. 5 (2002) 301-307
    • (2002) Nat. Neurosci. , vol.5 , pp. 301-307
    • Subramaniam, J.R.1
  • 19
    • 0036076642 scopus 로고    scopus 로고
    • Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site
    • Wang J., et al. Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol. Dis. 10 (2002) 128-138
    • (2002) Neurobiol. Dis. , vol.10 , pp. 128-138
    • Wang, J.1
  • 20
    • 31544467869 scopus 로고    scopus 로고
    • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models
    • Jonsson P.A., et al. Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models. Brain 129 (2005) 451-464
    • (2005) Brain , vol.129 , pp. 451-464
    • Jonsson, P.A.1
  • 21
    • 26444542945 scopus 로고    scopus 로고
    • Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: αB-crystallin modulates aggregation
    • Wang J., et al. Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: αB-crystallin modulates aggregation. Hum. Mol. Genet. 14 (2005) 2335-2347
    • (2005) Hum. Mol. Genet. , vol.14 , pp. 2335-2347
    • Wang, J.1
  • 22
    • 0031051485 scopus 로고    scopus 로고
    • ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
    • Bruijn L.I., et al. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron 18 (1997) 327-338
    • (1997) Neuron , vol.18 , pp. 327-338
    • Bruijn, L.I.1
  • 23
    • 0032544674 scopus 로고    scopus 로고
    • Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
    • Bruijn L.I., et al. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 281 (1998) 1851-1854
    • (1998) Science , vol.281 , pp. 1851-1854
    • Bruijn, L.I.1
  • 24
    • 0030777650 scopus 로고    scopus 로고
    • Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS
    • Durham H.D., et al. Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS. J. Neuropathol. Exp. Neurol. 56 (1997) 52-56
    • (1997) J. Neuropathol. Exp. Neurol. , vol.56 , pp. 52-56
    • Durham, H.D.1
  • 25
    • 0033749379 scopus 로고    scopus 로고
    • Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
    • Johnston J.F., et al. Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. U S A 97 (2000) 12571-12576
    • (2000) Proc. Natl. Acad. Sci. U S A , vol.97 , pp. 12571-12576
    • Johnston, J.F.1
  • 26
    • 0037707633 scopus 로고    scopus 로고
    • High threshold for induction of the stress response in motor neurons is associated with failure to activate HSF1
    • Batulan Z., et al. High threshold for induction of the stress response in motor neurons is associated with failure to activate HSF1. J. Neurosci. 23 (2003) 5789-5798
    • (2003) J. Neurosci. , vol.23 , pp. 5789-5798
    • Batulan, Z.1
  • 27
    • 0036892683 scopus 로고    scopus 로고
    • Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis
    • Urushitani M., et al. Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis. J. Neurochem. 83 (2002) 1030-1042
    • (2002) J. Neurochem. , vol.83 , pp. 1030-1042
    • Urushitani, M.1
  • 28
    • 3242701496 scopus 로고    scopus 로고
    • Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
    • Liu J., et al. Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron (2004) 435-517
    • (2004) Neuron , pp. 435-517
    • Liu, J.1
  • 29
    • 3242703300 scopus 로고    scopus 로고
    • Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria
    • Pasinelli P., et al. Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron 43 (2004) 19-30
    • (2004) Neuron , vol.43 , pp. 19-30
    • Pasinelli, P.1
  • 30
    • 0037068836 scopus 로고    scopus 로고
    • Motoneuron death triggered by a specific pathway downstream of Fas potentiation by ALS-linked SOD1 mutations
    • Raoul C., et al. Motoneuron death triggered by a specific pathway downstream of Fas potentiation by ALS-linked SOD1 mutations. Neuron 35 (2002) 1067-1083
    • (2002) Neuron , vol.35 , pp. 1067-1083
    • Raoul, C.1
  • 31
    • 33646452761 scopus 로고    scopus 로고
    • Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models
    • Bergemalm, et al. Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models. J. Neurosci. 26 (2006) 4147-4154
    • (2006) J. Neurosci. , vol.26 , pp. 4147-4154
    • Bergemalm1
  • 32
    • 33646466296 scopus 로고    scopus 로고
    • Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria
    • Deng, et al. Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc. Natl. Acad. Sci. U S A 103 (2006) 7142-7147
    • (2006) Proc. Natl. Acad. Sci. U S A , vol.103 , pp. 7142-7147
    • Deng1
  • 33
    • 29444443348 scopus 로고    scopus 로고
    • Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
    • Urushitani M., et al. Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat. Neurosci. 9 (2006) 108-118
    • (2006) Nat. Neurosci. , vol.9 , pp. 108-118
    • Urushitani, M.1
  • 34
    • 33645798615 scopus 로고    scopus 로고
    • Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model
    • Kikuchi H., et al. Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model. Proc. Natl. Acad. Sci. U S A 103 (2006) 6025-6030
    • (2006) Proc. Natl. Acad. Sci. U S A , vol.103 , pp. 6025-6030
    • Kikuchi, H.1
  • 35
    • 0141642203 scopus 로고    scopus 로고
    • Wild-type non-neuronal cells extend survival of SOD1 mutant motor neurons in ALS mice
    • Clement A.M., et al. Wild-type non-neuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 302 (2003) 113-117
    • (2003) Science , vol.302 , pp. 113-117
    • Clement, A.M.1
  • 36
    • 33744798774 scopus 로고    scopus 로고
    • Onset and progression in inherited ALS determined by motor neurons and microglia
    • Boillée S., et al. Onset and progression in inherited ALS determined by motor neurons and microglia. Science 312 (2006) 1389-1392
    • (2006) Science , vol.312 , pp. 1389-1392
    • Boillée, S.1
  • 37
    • 33750962014 scopus 로고    scopus 로고
    • Absence of TNF-alpha does not affect motor neuron disease caused by SOD1 mutations
    • Gowing G., et al. Absence of TNF-alpha does not affect motor neuron disease caused by SOD1 mutations. J. Neurosci. 26 (2006) 11397-11402
    • (2006) J. Neurosci. , vol.26 , pp. 11397-11402
    • Gowing, G.1
  • 38
    • 33745913017 scopus 로고    scopus 로고
    • Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin
    • Millecamps S., et al. Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin. J. Neurochem. 98 (2006) 926-938
    • (2006) J. Neurochem. , vol.98 , pp. 926-938
    • Millecamps, S.1
  • 39
    • 23844448652 scopus 로고    scopus 로고
    • Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress
    • Cai H., et al. Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress. J. Neurosci. 25 (2005) 7567-7574
    • (2005) J. Neurosci. , vol.25 , pp. 7567-7574
    • Cai, H.1
  • 40
    • 31144448704 scopus 로고    scopus 로고
    • Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking
    • Hadano S., et al. Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking. Hum. Mol. Genet. 15 (2006) 233-250
    • (2006) Hum. Mol. Genet. , vol.15 , pp. 233-250
    • Hadano, S.1
  • 41
    • 33745473350 scopus 로고    scopus 로고
    • Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities
    • Devon R.S., et al. Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities. Proc. Natl. Acad. Sci. U S A 103 (2006) 9595-9600
    • (2006) Proc. Natl. Acad. Sci. U S A , vol.103 , pp. 9595-9600
    • Devon, R.S.1
  • 42
    • 33745728401 scopus 로고    scopus 로고
    • Progressive spinal axonal degeneration and slowness in ALS2-deficient mice
    • Yamanaka K., et al. Progressive spinal axonal degeneration and slowness in ALS2-deficient mice. Ann. Neurol. 60 (2006) 95-104
    • (2006) Ann. Neurol. , vol.60 , pp. 95-104
    • Yamanaka, K.1
  • 43
    • 33751013740 scopus 로고    scopus 로고
    • Transgenic mouse models of amyotrophic lateral sclerosis
    • 10.1016/jbbadis.2006.03.006
    • Julien J.-P., and Kriz J. Transgenic mouse models of amyotrophic lateral sclerosis. Biochim. Biophys. Acta (2006) 10.1016/jbbadis.2006.03.006
    • (2006) Biochim. Biophys. Acta
    • Julien, J.-P.1    Kriz, J.2
  • 44
    • 0035369084 scopus 로고    scopus 로고
    • Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta
    • Zhao C., et al. Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta. Cell 105 (2001) 587-597
    • (2001) Cell , vol.105 , pp. 587-597
    • Zhao, C.1
  • 45
    • 0037437149 scopus 로고    scopus 로고
    • Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A
    • Xia C.H., et al. Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J. Cell Biol. 161 (2003) 55-66
    • (2003) J. Cell Biol. , vol.161 , pp. 55-66
    • Xia, C.H.1
  • 46
    • 0037198698 scopus 로고    scopus 로고
    • Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration
    • LaMonte B.H., et al. Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration. Neuron 34 (2002) 715-727
    • (2002) Neuron , vol.34 , pp. 715-727
    • LaMonte, B.H.1
  • 47
    • 0037734370 scopus 로고    scopus 로고
    • Mutations in dynein link motor neuron degeneration to defects in retrograde transport
    • Hafezparast M., et al. Mutations in dynein link motor neuron degeneration to defects in retrograde transport. Science 300 (2003) 808-812
    • (2003) Science , vol.300 , pp. 808-812
    • Hafezparast, M.1
  • 48
    • 21344472483 scopus 로고    scopus 로고
    • A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice
    • Dairin K., et al. A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J. Cell Biol. 169 (2005) 561-567
    • (2005) J. Cell Biol. , vol.169 , pp. 561-567
    • Dairin, K.1
  • 49
    • 0037175395 scopus 로고    scopus 로고
    • Missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy, a model of human motoneuron disease
    • Bommel H., et al. Missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy, a model of human motoneuron disease. J. Cell Biol. 159 (2002) 563-569
    • (2002) J. Cell Biol. , vol.159 , pp. 563-569
    • Bommel, H.1
  • 50
    • 0036842251 scopus 로고    scopus 로고
    • A missense mutation in Tbce causes progressive motor neuronopathy in mice
    • Martin N., et al. A missense mutation in Tbce causes progressive motor neuronopathy in mice. Nat. Genet. 32 (2002) 443-447
    • (2002) Nat. Genet. , vol.32 , pp. 443-447
    • Martin, N.1
  • 51
    • 0034978562 scopus 로고    scopus 로고
    • Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration
    • Oosthuyse B., et al. Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration. Nat. Genet. 28 (2001) 131-138
    • (2001) Nat. Genet. , vol.28 , pp. 131-138
    • Oosthuyse, B.1
  • 52
    • 0030050727 scopus 로고    scopus 로고
    • Benefit of vitamin E, riluzole and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis
    • Gurney M.E., et al. Benefit of vitamin E, riluzole and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis. Ann. Neurol. 39 (1996) 147-157
    • (1996) Ann. Neurol. , vol.39 , pp. 147-157
    • Gurney, M.E.1
  • 53
    • 0031931578 scopus 로고    scopus 로고
    • Riluzole preserves motor function in a transgenic model of familial amyotrophic lateral sclerosis
    • Gurney M.E., et al. Riluzole preserves motor function in a transgenic model of familial amyotrophic lateral sclerosis. Neurology 50 (1998) 62-66
    • (1998) Neurology , vol.50 , pp. 62-66
    • Gurney, M.E.1
  • 54
    • 0344609220 scopus 로고    scopus 로고
    • Beneficial effects of lysine acetylsalicylate, a soluble salt of aspirin, on motor performance in a transgenic model of amyotrophic lateral sclerosis
    • Barneoud P., and Curet O. Beneficial effects of lysine acetylsalicylate, a soluble salt of aspirin, on motor performance in a transgenic model of amyotrophic lateral sclerosis. Exp. Neurol. 155 (1999) 243-251
    • (1999) Exp. Neurol. , vol.155 , pp. 243-251
    • Barneoud, P.1    Curet, O.2
  • 55
    • 0036406903 scopus 로고    scopus 로고
    • Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis
    • Kriz J., et al. Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol. Dis. 10 (2002) 268-278
    • (2002) Neurobiol. Dis. , vol.10 , pp. 268-278
    • Kriz, J.1
  • 56
    • 0037007645 scopus 로고    scopus 로고
    • Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice
    • Zhu S., et al. Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice. Nature 417 (2002) 74-78
    • (2002) Nature , vol.417 , pp. 74-78
    • Zhu, S.1
  • 57
    • 0037067009 scopus 로고    scopus 로고
    • Minocycline delays disease onset and mortality in a transgenic model of ALS
    • Van Den Bosch L., et al. Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport 13 (2002) 1067-1070
    • (2002) Neuroreport , vol.13 , pp. 1067-1070
    • Van Den Bosch, L.1
  • 58
    • 0037381851 scopus 로고    scopus 로고
    • Efficient three-drug cocktail for disease induced by mutant superoxide dismutase
    • Kriz J., et al. Efficient three-drug cocktail for disease induced by mutant superoxide dismutase. Ann. Neurol. 53 (2003) 429-436
    • (2003) Ann. Neurol. , vol.53 , pp. 429-436
    • Kriz, J.1
  • 59
    • 19944428649 scopus 로고    scopus 로고
    • Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression
    • Rothstein J.D., et al. Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression. Nature 433 (2005) 73-77
    • (2005) Nature , vol.433 , pp. 73-77
    • Rothstein, J.D.1
  • 60
    • 23244439603 scopus 로고    scopus 로고
    • Manganese porphyrin given at symptom onset markedly extends survival of ALS mice
    • Crow J.P., et al. Manganese porphyrin given at symptom onset markedly extends survival of ALS mice. Ann. Neurol. 58 (2005) 258-265
    • (2005) Ann. Neurol. , vol.58 , pp. 258-265
    • Crow, J.P.1
  • 61
    • 16644382257 scopus 로고    scopus 로고
    • Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS
    • Storkebaum E., et al. Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS. Nat. Neurosc. 8 (2004) 85-92
    • (2004) Nat. Neurosc. , vol.8 , pp. 85-92
    • Storkebaum, E.1
  • 62
    • 0037104725 scopus 로고    scopus 로고
    • Neuroprotective effects of glial cell line-derived neurotrophic factor mediated by an adeno-associated virus vector in a transgenic animal model of amyotrophic lateral sclerosis
    • Wang L.J., et al. Neuroprotective effects of glial cell line-derived neurotrophic factor mediated by an adeno-associated virus vector in a transgenic animal model of amyotrophic lateral sclerosis. J. Neurosci. 22 (2002) 6920-6928
    • (2002) J. Neurosci. , vol.22 , pp. 6920-6928
    • Wang, L.J.1
  • 63
    • 0042528664 scopus 로고    scopus 로고
    • Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model
    • Kaspar B.K., et al. Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model. Science 301 (2003) 839-842
    • (2003) Science , vol.301 , pp. 839-842
    • Kaspar, B.K.1
  • 64
    • 2642526164 scopus 로고    scopus 로고
    • VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model
    • Azzouz M., et al. VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model. Nature 429 (2004) 413-417
    • (2004) Nature , vol.429 , pp. 413-417
    • Azzouz, M.1
  • 65
    • 20244381261 scopus 로고    scopus 로고
    • Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model
    • Ralph G.S., et al. Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model. Nat. Med. 11 (2005) 429-433
    • (2005) Nat. Med. , vol.11 , pp. 429-433
    • Ralph, G.S.1
  • 66
    • 17644383664 scopus 로고    scopus 로고
    • Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS
    • Raoul C., et al. Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS. Nat. Med. 11 (2005) 423-428
    • (2005) Nat. Med. , vol.11 , pp. 423-428
    • Raoul, C.1
  • 67
    • 18244396929 scopus 로고    scopus 로고
    • Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis
    • Miller T.M., et al. Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis. Ann. Neurol. 57 (2005) 773-776
    • (2005) Ann. Neurol. , vol.57 , pp. 773-776
    • Miller, T.M.1
  • 68
    • 33846643867 scopus 로고    scopus 로고
    • Urushitani, M. et al. (2007) Therapeutic effects of immunization with mutant superoxide dismutase in mice models of ALS. Proc. Natl. Acad. Sci. USA, in press.
  • 69
    • 0033051815 scopus 로고    scopus 로고
    • Neuroprotective effects of creatin in a transgenic animal model of amyotrophic lateral sclerosis
    • Klivenyi P., et al. Neuroprotective effects of creatin in a transgenic animal model of amyotrophic lateral sclerosis. Nat. Med. 5 (1999) 347-350
    • (1999) Nat. Med. , vol.5 , pp. 347-350
    • Klivenyi, P.1
  • 70
    • 0037379650 scopus 로고    scopus 로고
    • A randomized sequential trial of creatine monohydrate in amyotrophic lateral sclerosis
    • Groeneveld G.J., et al. A randomized sequential trial of creatine monohydrate in amyotrophic lateral sclerosis. Ann. Neurol. 53 (2003) 437-445
    • (2003) Ann. Neurol. , vol.53 , pp. 437-445
    • Groeneveld, G.J.1
  • 71
    • 0036895241 scopus 로고    scopus 로고
    • Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS
    • Drachman D.B., et al. Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS. Ann. Neurol. 52 (2002) 771-778
    • (2002) Ann. Neurol. , vol.52 , pp. 771-778
    • Drachman, D.B.1
  • 72
    • 33745728411 scopus 로고    scopus 로고
    • Trial of celecoxib in amyotrophic lateral sclerosis
    • Cudkowicz M.E., et al. Trial of celecoxib in amyotrophic lateral sclerosis. Ann. Neurol. 60 (2006) 22-31
    • (2006) Ann. Neurol. , vol.60 , pp. 22-31
    • Cudkowicz, M.E.1
  • 73
    • 33745686121 scopus 로고    scopus 로고
    • Recovery from paralysis in adult rats using embryonic stem cells
    • Deshpande D.M., et al. Recovery from paralysis in adult rats using embryonic stem cells. Ann. Neurol. 60 (2006) 32-44
    • (2006) Ann. Neurol. , vol.60 , pp. 32-44
    • Deshpande, D.M.1
  • 74
    • 0027465098 scopus 로고
    • Progressive Neuronopathy in Transgenic Mice Expressing the Human Heavy Neurofilament (NFH) Gene: A Mouse Model of Amyotrophic Lateral Sclerosis
    • Côté F., et al. Progressive Neuronopathy in Transgenic Mice Expressing the Human Heavy Neurofilament (NFH) Gene: A Mouse Model of Amyotrophic Lateral Sclerosis. Cell 73 (1993) 35-46
    • (1993) Cell , vol.73 , pp. 35-46
    • Côté, F.1
  • 75
    • 0028116467 scopus 로고
    • A mutant neurofilament subunit causes massive, selective motor neuron death: implications for the pathogenesis of human motor neuron disease
    • Lee M.K., et al. A mutant neurofilament subunit causes massive, selective motor neuron death: implications for the pathogenesis of human motor neuron disease. Neuron 13 (1994) 975-988
    • (1994) Neuron , vol.13 , pp. 975-988
    • Lee, M.K.1
  • 76
    • 17744368458 scopus 로고    scopus 로고
    • Deregulation of Cdk5 in a mouse model of ALS: Toxicity alleviated by perikaryal neurofilament inclusions
    • Nguyen M.D., et al. Deregulation of Cdk5 in a mouse model of ALS: Toxicity alleviated by perikaryal neurofilament inclusions. Neuron 30 (2001) 135-147
    • (2001) Neuron , vol.30 , pp. 135-147
    • Nguyen, M.D.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.