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Volumn 4, Issue C, 2004, Pages 169-186

Genetics of amyotrophic lateral sclerosis

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EID: 77957099429     PISSN: 15674231     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S1567-4231(04)04008-0     Document Type: Article
Times cited : (5)

References (175)
  • 1
    • 0028971936 scopus 로고
    • Induction of nitrotyrosine-like immunoreactivity in the lower motor neuron of amyotrophic lateral sclerosis
    • Abe K., Pan L.H., Watanabe M., Kato T., and Itoyama Y. Induction of nitrotyrosine-like immunoreactivity in the lower motor neuron of amyotrophic lateral sclerosis. Neurosci. Lett 199 (1995) 152-154
    • (1995) Neurosci. Lett , vol.199 , pp. 152-154
    • Abe, K.1    Pan, L.H.2    Watanabe, M.3    Kato, T.4    Itoyama, Y.5
  • 13
    • 0031784348 scopus 로고    scopus 로고
    • Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis
    • Andrus P.K., Fleck T.J., Gurney M.E., and Hall E.D. Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis. J. Neurochem 71 (1998) 2041-2048
    • (1998) J. Neurochem , vol.71 , pp. 2041-2048
    • Andrus, P.K.1    Fleck, T.J.2    Gurney, M.E.3    Hall, E.D.4
  • 15
    • 0028168971 scopus 로고
    • Familial amyotrophic lateral sclerosis (ALS) in Japan associated with H46R mutation in Cu/Zn superoxide dismutase gene: a possible new subtype of familial ALS
    • Aoki M., Ogasawara M., Matsubara Y., Narisawa K., Nakamura S., Itoyama Y., and Abe K. Familial amyotrophic lateral sclerosis (ALS) in Japan associated with H46R mutation in Cu/Zn superoxide dismutase gene: a possible new subtype of familial ALS. J. Neurol. Sci 126 (1994) 77-83
    • (1994) J. Neurol. Sci , vol.126 , pp. 77-83
    • Aoki, M.1    Ogasawara, M.2    Matsubara, Y.3    Narisawa, K.4    Nakamura, S.5    Itoyama, Y.6    Abe, K.7
  • 16
    • 0031957298 scopus 로고    scopus 로고
    • Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis
    • Aoki M., Lin C.L., Rothstein J.D., Geller B.A., Hosler B.A., Munsat T.L., Horvitz H.R., and Brown Jr. R.H. Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis. Ann. Neurol 43 (1998) 645-653
    • (1998) Ann. Neurol , vol.43 , pp. 645-653
    • Aoki, M.1    Lin, C.L.2    Rothstein, J.D.3    Geller, B.A.4    Hosler, B.A.5    Munsat, T.L.6    Horvitz, H.R.7    Brown Jr., R.H.8
  • 17
    • 0035856861 scopus 로고    scopus 로고
    • Leukemia inhibitory factor by systemic administration rescues spinal motor neurons in the SOD1 G93A murine model of familial amyotrophic lateral sclerosis
    • Azari M.F., Galle A., Lopes E.C., Kurek J., and Cheema S.S. Leukemia inhibitory factor by systemic administration rescues spinal motor neurons in the SOD1 G93A murine model of familial amyotrophic lateral sclerosis. Brain Res 922 (2001) 144-147
    • (2001) Brain Res , vol.922 , pp. 144-147
    • Azari, M.F.1    Galle, A.2    Lopes, E.C.3    Kurek, J.4    Cheema, S.S.5
  • 21
    • 0025237394 scopus 로고
    • Hereditary motor system diseases (chronic juvenile amyotrophic lateral sclerosis). Conditions combining a bilateral pyramidal syndrome with limb and bulbar amyotrophy
    • Ben Hamida M., Hentati F., and Ben Hamida C. Hereditary motor system diseases (chronic juvenile amyotrophic lateral sclerosis). Conditions combining a bilateral pyramidal syndrome with limb and bulbar amyotrophy. Brain 113 (1990) 347-363
    • (1990) Brain , vol.113 , pp. 347-363
    • Ben Hamida, M.1    Hentati, F.2    Ben Hamida, C.3
  • 23
    • 0031868461 scopus 로고    scopus 로고
    • Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis
    • Bogdanov M.B., Ramos L.E., Xu Z., and Beal M.F. Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis. J. Neurochem 71 (1998) 1321-1324
    • (1998) J. Neurochem , vol.71 , pp. 1321-1324
    • Bogdanov, M.B.1    Ramos, L.E.2    Xu, Z.3    Beal, M.F.4
  • 24
    • 0037059040 scopus 로고    scopus 로고
    • Chaperoning brain degeneration
    • Bonini N.M. Chaperoning brain degeneration. Proc. Natl Acad. Sci. USA 99 Suppl. 4 (2002) 16407-16411
    • (2002) Proc. Natl Acad. Sci. USA , vol.99 , Issue.SUPPL. 4 , pp. 16407-16411
    • Bonini, N.M.1
  • 27
    • 0027359334 scopus 로고
    • Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis
    • Bowling A.C., Schulz J.B., Brown Jr. R.H., and Beal M.F. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis. J. Neurochem 61 (1993) 2322-2325
    • (1993) J. Neurochem , vol.61 , pp. 2322-2325
    • Bowling, A.C.1    Schulz, J.B.2    Brown Jr., R.H.3    Beal, M.F.4
  • 29
    • 0034961104 scopus 로고    scopus 로고
    • Amyotrophic lateral sclerosis: pathogenesis
    • Brown Jr. R.H., and Robberecht W. Amyotrophic lateral sclerosis: pathogenesis. Semin. Neurol 21 (2001) 131-139
    • (2001) Semin. Neurol , vol.21 , pp. 131-139
    • Brown Jr., R.H.1    Robberecht, W.2
  • 30
    • 0344507132 scopus 로고    scopus 로고
    • Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis
    • Bruening W., Roy J., Giasson B., Figlewicz D.A., Mushynski W.E., and Durham H.D. Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis. J. Neurochem 72 (1999) 693-699
    • (1999) J. Neurochem , vol.72 , pp. 693-699
    • Bruening, W.1    Roy, J.2    Giasson, B.3    Figlewicz, D.A.4    Mushynski, W.E.5    Durham, H.D.6
  • 31
    • 0030806228 scopus 로고    scopus 로고
    • Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant
    • Bruijn L.I., Beal M.F., Becher M.W., Schulz J.B., Wong P.C., Price D.L., and Cleveland D.W. Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant. Proc. Natl Acad. Sci. USA 94 (1997) 7606-7611
    • (1997) Proc. Natl Acad. Sci. USA , vol.94 , pp. 7606-7611
    • Bruijn, L.I.1    Beal, M.F.2    Becher, M.W.3    Schulz, J.B.4    Wong, P.C.5    Price, D.L.6    Cleveland, D.W.7
  • 36
    • 0023221049 scopus 로고
    • Phenotypic and genotypic heterogeneity of dominantly inherited amyotrophic lateral sclerosis
    • Chio A., Brignolio F., Meineri P., and Schiffer D. Phenotypic and genotypic heterogeneity of dominantly inherited amyotrophic lateral sclerosis. Acta. Neurol. Scand 75 (1987) 277-282
    • (1987) Acta. Neurol. Scand , vol.75 , pp. 277-282
    • Chio, A.1    Brignolio, F.2    Meineri, P.3    Schiffer, D.4
  • 37
    • 0035516124 scopus 로고    scopus 로고
    • From charcot to lou gehrig: deciphering selective motor neuron death in als
    • Cleveland D.W., and Rothstein J.D. From charcot to lou gehrig: deciphering selective motor neuron death in als. Nat. Rev. Neurosci 2 (2001) 806-819
    • (2001) Nat. Rev. Neurosci , vol.2 , pp. 806-819
    • Cleveland, D.W.1    Rothstein, J.D.2
  • 41
    • 0027465098 scopus 로고
    • Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis
    • Cote F., Collard J.F., and Julien J.P. Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis. Cell 73 (1993) 35-46
    • (1993) Cell , vol.73 , pp. 35-46
    • Cote, F.1    Collard, J.F.2    Julien, J.P.3
  • 42
    • 0032483016 scopus 로고    scopus 로고
    • Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
    • Couillard-Despres S., Zhu Q., Wong P.C., Price D.L., Cleveland D.W., and Julien J.P. Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase. Proc. Natl Acad. Sci. USA 95 (1998) 9626-9630
    • (1998) Proc. Natl Acad. Sci. USA , vol.95 , pp. 9626-9630
    • Couillard-Despres, S.1    Zhu, Q.2    Wong, P.C.3    Price, D.L.4    Cleveland, D.W.5    Julien, J.P.6
  • 43
    • 0030833449 scopus 로고    scopus 로고
    • Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite
    • Crow J.P., Sampson J.B., Zhuang Y., Thompson J.A., and Beckman J.S. Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite. J. Neurochem 69 (1997) 1936-1944
    • (1997) J. Neurochem , vol.69 , pp. 1936-1944
    • Crow, J.P.1    Sampson, J.B.2    Zhuang, Y.3    Thompson, J.A.4    Beckman, J.S.5
  • 45
    • 0035394668 scopus 로고    scopus 로고
    • Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
    • Cummings C.J., Sun Y., Opal P., Antalffy B., Mestril R., Orr H.T., Dillmann W.H., and Zoghbi H.Y. Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum. Mol. Genet 10 (2001) 1511-1518
    • (2001) Hum. Mol. Genet , vol.10 , pp. 1511-1518
    • Cummings, C.J.1    Sun, Y.2    Opal, P.3    Antalffy, B.4    Mestril, R.5    Orr, H.T.6    Dillmann, W.H.7    Zoghbi, H.Y.8
  • 47
    • 0042914405 scopus 로고    scopus 로고
    • The first non-sense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings
    • Devon R.S., Helm J.R., Rouleau G.A., Leitner Y., Lerman-Sagie T., Lev D., and Hayden M.R. The first non-sense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings. Clin. Genet 64 (2003) 210-215
    • (2003) Clin. Genet , vol.64 , pp. 210-215
    • Devon, R.S.1    Helm, J.R.2    Rouleau, G.A.3    Leitner, Y.4    Lerman-Sagie, T.5    Lev, D.6    Hayden, M.R.7
  • 48
    • 0030777650 scopus 로고    scopus 로고
    • Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS
    • Durham H.D., Roy J., Dong L., and Figlewicz D.A. Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS. J. Neuropathol. Exp. Neurol 56 (1997) 523-530
    • (1997) J. Neuropathol. Exp. Neurol , vol.56 , pp. 523-530
    • Durham, H.D.1    Roy, J.2    Dong, L.3    Figlewicz, D.A.4
  • 54
    • 0028001606 scopus 로고
    • Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis
    • Figlewicz D.A., Krizus A., Martinoli M.G., Meininger V., Dib M., Rouleau G.A., and Julien J.P. Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis. Hum. Mol. Genet 3 (1994) 1757-1761
    • (1994) Hum. Mol. Genet , vol.3 , pp. 1757-1761
    • Figlewicz, D.A.1    Krizus, A.2    Martinoli, M.G.3    Meininger, V.4    Dib, M.5    Rouleau, G.A.6    Julien, J.P.7
  • 55
    • 0035020941 scopus 로고    scopus 로고
    • Intron 7 retention and exon 9 skipping EAAT2 mRNA variants are not associated with amyotrophic lateral sclerosis
    • Flowers J.M., Powell J.F., Leigh P.N., Andersen P., and Shaw C.E. Intron 7 retention and exon 9 skipping EAAT2 mRNA variants are not associated with amyotrophic lateral sclerosis. Ann. Neurol 49 (2001) 643-649
    • (2001) Ann. Neurol , vol.49 , pp. 643-649
    • Flowers, J.M.1    Powell, J.F.2    Leigh, P.N.3    Andersen, P.4    Shaw, C.E.5
  • 56
    • 0015935503 scopus 로고
    • On the stability of bovine superoxide dismutase. The effects of metals
    • Forman H.J., and Fridovich I. On the stability of bovine superoxide dismutase. The effects of metals. J. Biol. Chem 248 (1973) 2645-2649
    • (1973) J. Biol. Chem , vol.248 , pp. 2645-2649
    • Forman, H.J.1    Fridovich, I.2
  • 57
    • 0031879593 scopus 로고    scopus 로고
    • The expression of the glial glutamate transporter protein EAAT2 in motor neuron disease: an immunohistochemical study
    • Fray A.E., Ince P.G., Banner S.J., Milton I.D., Usher P.A., Cookson M.R., and Shaw P.J. The expression of the glial glutamate transporter protein EAAT2 in motor neuron disease: an immunohistochemical study. Eur. J. Neurosci 10 (1998) 2481-2489
    • (1998) Eur. J. Neurosci , vol.10 , pp. 2481-2489
    • Fray, A.E.1    Ince, P.G.2    Banner, S.J.3    Milton, I.D.4    Usher, P.A.5    Cookson, M.R.6    Shaw, P.J.7
  • 59
    • 0035503911 scopus 로고    scopus 로고
    • Kennedy's disease: a triplet repeat disorder or a motor neuron disease?
    • Gallo J.M. Kennedy's disease: a triplet repeat disorder or a motor neuron disease?. Brain Res. Bull 56 (2001) 209-214
    • (2001) Brain Res. Bull , vol.56 , pp. 209-214
    • Gallo, J.M.1
  • 60
    • 0034701007 scopus 로고    scopus 로고
    • Potential role of LIF as a modifier gene in the pathogenesis of amyotrophic lateral sclerosis
    • Giess R., Beck M., Goetz R., Nitsch R.M., Toyka K.V., and Sendtner M. Potential role of LIF as a modifier gene in the pathogenesis of amyotrophic lateral sclerosis. Neurology 54 (2000) 1003-1005
    • (2000) Neurology , vol.54 , pp. 1003-1005
    • Giess, R.1    Beck, M.2    Goetz, R.3    Nitsch, R.M.4    Toyka, K.V.5    Sendtner, M.6
  • 66
    • 0032127330 scopus 로고    scopus 로고
    • Relationship of oxygen radical-induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS
    • Hall E.D., Andrus P.K., Oostveen J.A., Fleck T.J., and Gurney M.E. Relationship of oxygen radical-induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS. J. Neurosci. Res 53 (1998) 66-77
    • (1998) J. Neurosci. Res , vol.53 , pp. 66-77
    • Hall, E.D.1    Andrus, P.K.2    Oostveen, J.A.3    Fleck, T.J.4    Gurney, M.E.5
  • 67
    • 0035136084 scopus 로고    scopus 로고
    • Compound heterozygous D90A and D96N SOD1 mutations in a recessive amyotrophic lateral sclerosis family
    • Hand C.K., Mayeux-Portas V., Khoris J., Briolotti V., Clavelou P., Camu W., and Rouleau G.A. Compound heterozygous D90A and D96N SOD1 mutations in a recessive amyotrophic lateral sclerosis family. Ann. Neurol 49 (2001) 267-271
    • (2001) Ann. Neurol , vol.49 , pp. 267-271
    • Hand, C.K.1    Mayeux-Portas, V.2    Khoris, J.3    Briolotti, V.4    Clavelou, P.5    Camu, W.6    Rouleau, G.A.7
  • 70
    • 0029037348 scopus 로고
    • Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction
    • Haverkamp L.J., Appel V., and Appel S.H. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 118 (1995) 707-719
    • (1995) Brain , vol.118 , pp. 707-719
    • Haverkamp, L.J.1    Appel, V.2    Appel, S.H.3
  • 71
    • 0033009789 scopus 로고    scopus 로고
    • Molecular genetic analysis of the APEX nuclease gene in amyotrophic lateral sclerosis
    • Hayward C., Colville S., Swingler R.J., and Brock D.J. Molecular genetic analysis of the APEX nuclease gene in amyotrophic lateral sclerosis. Neurology 52 (1999) 1899-1901
    • (1999) Neurology , vol.52 , pp. 1899-1901
    • Hayward, C.1    Colville, S.2    Swingler, R.J.3    Brock, D.J.4
  • 74
    • 0014063240 scopus 로고
    • Familial amyotrophic lateral sclerosis. A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells
    • Hirano A., Kurland L.T., and Sayre G.P. Familial amyotrophic lateral sclerosis. A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells. Arch. Neurol 16 (1967) 232-243
    • (1967) Arch. Neurol , vol.16 , pp. 232-243
    • Hirano, A.1    Kurland, L.T.2    Sayre, G.P.3
  • 78
    • 0018836252 scopus 로고
    • Hereditary amyotrophic lateral sclerosis transmitted for five generations
    • Husquinet H., and Franck G. Hereditary amyotrophic lateral sclerosis transmitted for five generations. Clin. Genet 18 (1980) 109-115
    • (1980) Clin. Genet , vol.18 , pp. 109-115
    • Husquinet, H.1    Franck, G.2
  • 80
    • 0031888254 scopus 로고    scopus 로고
    • Genetic variation in the ciliary neurotrophic factor receptor alpha gene and familial amyotrophic lateral sclerosis
    • Imura T., Shimohama S., Kawamata J., and Kimura J. Genetic variation in the ciliary neurotrophic factor receptor alpha gene and familial amyotrophic lateral sclerosis. Ann. Neurol 43 (1998) 275
    • (1998) Ann. Neurol , vol.43 , pp. 275
    • Imura, T.1    Shimohama, S.2    Kawamata, J.3    Kimura, J.4
  • 82
    • 0029943383 scopus 로고    scopus 로고
    • Analysis of chromosome 5q13 genes in amyotrophic lateral sclerosis: homozygous NAIP deletion in a sporadic case
    • Jackson M., Morrison K.E., Al-Chalabi A., Bakker M., and Leigh P.N. Analysis of chromosome 5q13 genes in amyotrophic lateral sclerosis: homozygous NAIP deletion in a sporadic case. Ann. Neurol 39 (1996) 796-800
    • (1996) Ann. Neurol , vol.39 , pp. 796-800
    • Jackson, M.1    Morrison, K.E.2    Al-Chalabi, A.3    Bakker, M.4    Leigh, P.N.5
  • 83
    • 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.A., Dalton M.J., Gurney M.E., and Kopito R.R. 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. USA 97 (2000) 12571-12576
    • (2000) Proc. Natl Acad. Sci. USA , vol.97 , pp. 12571-12576
    • Johnston, J.A.1    Dalton, M.J.2    Gurney, M.E.3    Kopito, R.R.4
  • 85
    • 0031015422 scopus 로고    scopus 로고
    • Prognosis in familial amyotrophic lateral sclerosis: progression and survival in patients with glu100gly and ala4val mutations in Cu,Zn superoxide dismutase
    • Juneja T., Pericak-Vance M.A., Laing N.G., Dave S., and Siddique T. Prognosis in familial amyotrophic lateral sclerosis: progression and survival in patients with glu100gly and ala4val mutations in Cu,Zn superoxide dismutase. Neurology 48 (1997) 55-57
    • (1997) Neurology , vol.48 , pp. 55-57
    • Juneja, T.1    Pericak-Vance, M.A.2    Laing, N.G.3    Dave, S.4    Siddique, T.5
  • 86
    • 0031458389 scopus 로고    scopus 로고
    • Prominent sensory and autonomic disturbances in familial amyotrophic lateral sclerosis with a Gly93Ser mutation in the SOD1 gene
    • Kawata A., Kato S., Hayashi H., and Hirai S. Prominent sensory and autonomic disturbances in familial amyotrophic lateral sclerosis with a Gly93Ser mutation in the SOD1 gene. J. Neurol. Sci 153 (1997) 82-85
    • (1997) J. Neurol. Sci , vol.153 , pp. 82-85
    • Kawata, A.1    Kato, S.2    Hayashi, H.3    Hirai, S.4
  • 87
    • 0030916372 scopus 로고    scopus 로고
    • Evidence of reduced DNA repair in amyotrophic lateral sclerosis brain tissue
    • Kisby G.E., Milne J., and Sweatt C. Evidence of reduced DNA repair in amyotrophic lateral sclerosis brain tissue. Neuroreport 8 (1997) 1337-1340
    • (1997) Neuroreport , vol.8 , pp. 1337-1340
    • Kisby, G.E.1    Milne, J.2    Sweatt, C.3
  • 88
    • 0034708793 scopus 로고    scopus 로고
    • Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract
    • Kobayashi Y., Kume A., Li M., Doyu M., Hata M., Ohtsuka K., and Sobue G. Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract. J. Biol. Chem 275 (2000) 8772-8778
    • (2000) J. Biol. Chem , vol.275 , pp. 8772-8778
    • Kobayashi, Y.1    Kume, A.2    Li, M.3    Doyu, M.4    Hata, M.5    Ohtsuka, K.6    Sobue, G.7
  • 89
    • 0032079517 scopus 로고    scopus 로고
    • Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1
    • Kong J., and Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J. Neurosci 18 (1998) 3241-3250
    • (1998) J. Neurosci , vol.18 , pp. 3241-3250
    • Kong, J.1    Xu, Z.2
  • 90
    • 0033953140 scopus 로고    scopus 로고
    • Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis
    • Kong J., and Xu Z. Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis. Neurosci. Lett 281 (2000) 72-74
    • (2000) Neurosci. Lett , vol.281 , pp. 72-74
    • Kong, J.1    Xu, Z.2
  • 91
    • 0034652127 scopus 로고    scopus 로고
    • Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins
    • Krobitsch S., and Lindquist S. Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins. Proc. Natl Acad. Sci. USA 97 (2000) 1589-1594
    • (2000) Proc. Natl Acad. Sci. USA , vol.97 , pp. 1589-1594
    • Krobitsch, S.1    Lindquist, S.2
  • 92
    • 0032731637 scopus 로고    scopus 로고
    • ALS-linked Cu/Zn-SOD mutation increases vulnerability of motor neurons to excitotoxicity by a mechanism involving increased oxidative stress and perturbed calcium homeostasis
    • Kruman I.I., Pedersen W.A., Springer J.E., and Mattson M.P. ALS-linked Cu/Zn-SOD mutation increases vulnerability of motor neurons to excitotoxicity by a mechanism involving increased oxidative stress and perturbed calcium homeostasis. Exp. Neurol 160 (1999) 28-39
    • (1999) Exp. Neurol , vol.160 , pp. 28-39
    • Kruman, I.I.1    Pedersen, W.A.2    Springer, J.E.3    Mattson, M.P.4
  • 97
    • 0023918762 scopus 로고
    • Comparison of sporadic and familial disease amongst 580 cases of motor neuron disease
    • Li T.M., Alberman E., and Swash M. Comparison of sporadic and familial disease amongst 580 cases of motor neuron disease. J. Neurol. Neurosurg. Psychiatry 51 (1988) 778-784
    • (1988) J. Neurol. Neurosurg. Psychiatry , vol.51 , pp. 778-784
    • Li, T.M.1    Alberman, E.2    Swash, M.3
  • 99
    • 0030749689 scopus 로고    scopus 로고
    • Normal binding and reactivity of copper in mutant superoxide dismutase isolated from amyotrophic lateral sclerosis patients
    • Marklund S., Andersen P., Forsgren L., Nilsson L., Wikander G., and Öberg A. Normal binding and reactivity of copper in mutant superoxide dismutase isolated from amyotrophic lateral sclerosis patients. J. Neurochem 69 (1997) 675-681
    • (1997) J. Neurochem , vol.69 , pp. 675-681
    • Marklund, S.1    Andersen, P.2    Forsgren, L.3    Nilsson, L.4    Wikander, G.5    Öberg, A.6
  • 100
    • 0027484249 scopus 로고
    • Disruption of the CNTF gene results in motor neuron degeneration
    • Masu Y., Wolf E., Holtmann B., Sendtner M., Brem G., and Thoenen H. Disruption of the CNTF gene results in motor neuron degeneration. Nature 365 (1993) 27-32
    • (1993) Nature , vol.365 , pp. 27-32
    • Masu, Y.1    Wolf, E.2    Holtmann, B.3    Sendtner, M.4    Brem, G.5    Thoenen, H.6
  • 101
    • 0031958077 scopus 로고    scopus 로고
    • Association between centromeric deletions of the SMN gene and sporadic adult-onset lower motor neuron disease
    • Moulard B., Salachas F., Chassande B., Briolotti V., Meininger V., Malafosse A., and Camu W. Association between centromeric deletions of the SMN gene and sporadic adult-onset lower motor neuron disease. Ann. Neurol 43 (1998) 640-644
    • (1998) Ann. Neurol , vol.43 , pp. 640-644
    • Moulard, B.1    Salachas, F.2    Chassande, B.3    Briolotti, V.4    Meininger, V.5    Malafosse, A.6    Camu, W.7
  • 102
    • 0028991091 scopus 로고
    • Apolipoprotein E epsilon 4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis
    • Mui S., Rebeck G.W., McKenna-Yasek D., Hyman B.T., and Brown Jr. R.H. Apolipoprotein E epsilon 4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis. Ann. Neurol 38 (1995) 460-463
    • (1995) Ann. Neurol , vol.38 , pp. 460-463
    • Mui, S.1    Rebeck, G.W.2    McKenna-Yasek, D.3    Hyman, B.T.4    Brown Jr., R.H.5
  • 103
    • 0022443737 scopus 로고
    • Familial adult motor neuron disease: amyotrophic lateral sclerosis
    • Mulder D.W., Kurland L.T., Offord K.P., and Beard C.M. Familial adult motor neuron disease: amyotrophic lateral sclerosis. Neurology 36 (1986) 511-517
    • (1986) Neurology , vol.36 , pp. 511-517
    • Mulder, D.W.1    Kurland, L.T.2    Offord, K.P.3    Beard, C.M.4
  • 104
    • 0032567807 scopus 로고    scopus 로고
    • Mutant AP endonuclease in patients with amyotrophic lateral sclerosis
    • Olkowski Z.L. Mutant AP endonuclease in patients with amyotrophic lateral sclerosis. Neuroreport 9 (1998) 239-242
    • (1998) Neuroreport , vol.9 , pp. 239-242
    • Olkowski, Z.L.1
  • 106
    • 0035838427 scopus 로고    scopus 로고
    • Clinical implications of the genetics of ALS and other motor neuron diseases
    • Orrell R.W., and Figlewicz D.A. Clinical implications of the genetics of ALS and other motor neuron diseases. Neurology 57 (2001) 9-17
    • (2001) Neurology , vol.57 , pp. 9-17
    • Orrell, R.W.1    Figlewicz, D.A.2
  • 108
    • 0031443762 scopus 로고    scopus 로고
    • Familial ALS is associated with mutations in all exons of SOD1: a novel mutation in exon 3 (Gly72Ser)
    • Orrell R.W., Marklund S.L., and deBelleroche J.S. Familial ALS is associated with mutations in all exons of SOD1: a novel mutation in exon 3 (Gly72Ser). J. Neurol. Sci 153 (1997) 46-49
    • (1997) J. Neurol. Sci , vol.153 , pp. 46-49
    • Orrell, R.W.1    Marklund, S.L.2    deBelleroche, J.S.3
  • 109
    • 0030899577 scopus 로고    scopus 로고
    • The relationship of spinal muscular atrophy to motor neuron disease: investigation of SMN and NAIP gene deletions in sporadic and familial ALS
    • Orrell R.W., Habgood J.J., de Belleroche J.S., and Lane R.J. The relationship of spinal muscular atrophy to motor neuron disease: investigation of SMN and NAIP gene deletions in sporadic and familial ALS. J. Neurol. Sci 145 (1997) 55-61
    • (1997) J. Neurol. Sci , vol.145 , pp. 55-61
    • Orrell, R.W.1    Habgood, J.J.2    de Belleroche, J.S.3    Lane, R.J.4
  • 110
    • 0030945491 scopus 로고    scopus 로고
    • Clinical and functional investigation of 10 missense mutations and a novel frameshift insertion mutation of the gene for copper-zinc superoxide dismutase in UK families with amyotrophic lateral sclerosis
    • Orrell R.W., Habgood J.J., Gardiner I., King A.W., Bowe F.A., Hallewell R.A., Marklund S.L., Greenwood J., Lane R.J., and deBelleroche J. Clinical and functional investigation of 10 missense mutations and a novel frameshift insertion mutation of the gene for copper-zinc superoxide dismutase in UK families with amyotrophic lateral sclerosis. Neurology 48 (1997) 746-751
    • (1997) Neurology , vol.48 , pp. 746-751
    • Orrell, R.W.1    Habgood, J.J.2    Gardiner, I.3    King, A.W.4    Bowe, F.A.5    Hallewell, R.A.6    Marklund, S.L.7    Greenwood, J.8    Lane, R.J.9    deBelleroche, J.10
  • 112
  • 113
    • 0028815433 scopus 로고
    • Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons
    • Pardo C.A., Xu Z., Borchelt D.R., Price D.L., Sisodia S.S., and Cleveland D.W. Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons. Proc. Natl Acad. Sci. USA 92 (1995) 954-958
    • (1995) Proc. Natl Acad. Sci. USA , vol.92 , pp. 954-958
    • Pardo, C.A.1    Xu, Z.2    Borchelt, D.R.3    Price, D.L.4    Sisodia, S.S.5    Cleveland, D.W.6
  • 114
    • 0036885011 scopus 로고    scopus 로고
    • D90A-SOD1 mediated amyotrophic lateral sclerosis: a single founder for all cases with evidence for a Cis-acting disease modifier in the recessive haplotype
    • Parton M.J., Broom W., Andersen P.M., Al-Chalabi A., Nigel Leigh P., Powell J.F., and Shaw C.E. D90A-SOD1 mediated amyotrophic lateral sclerosis: a single founder for all cases with evidence for a Cis-acting disease modifier in the recessive haplotype. Hum. Mutat 20 (2002) 473
    • (2002) Hum. Mutat , vol.20 , pp. 473
    • Parton, M.J.1    Broom, W.2    Andersen, P.M.3    Al-Chalabi, A.4    Nigel Leigh, P.5    Powell, J.F.6    Shaw, C.E.7
  • 115
    • 0031768026 scopus 로고    scopus 로고
    • Protein modification by the lipid peroxidation product 4-hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients
    • Pedersen W.A., Fu W., Keller J.N., Markesbery W.R., Appel S., Smith R.G., Kasarskis E., and Mattson M.P. Protein modification by the lipid peroxidation product 4-hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients. Ann. Neurol 44 (1998) 819-824
    • (1998) Ann. Neurol , vol.44 , pp. 819-824
    • Pedersen, W.A.1    Fu, W.2    Keller, J.N.3    Markesbery, W.R.4    Appel, S.5    Smith, R.G.6    Kasarskis, E.7    Mattson, M.P.8
  • 118
    • 0030450004 scopus 로고    scopus 로고
    • Cu/Zn superoxide dismutase gene mutations in amyotrophic lateral sclerosis: correlation between genotype and clinical features
    • Radunovic A., and Leigh P.N. Cu/Zn superoxide dismutase gene mutations in amyotrophic lateral sclerosis: correlation between genotype and clinical features. J. Neurol. Neurosurg. Psychiatry 61 (1996) 565-572
    • (1996) J. Neurol. Neurosurg. Psychiatry , vol.61 , pp. 565-572
    • Radunovic, A.1    Leigh, P.N.2
  • 121
    • 0028888945 scopus 로고
    • Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis
    • Ripps M.E., Huntley G.W., Hof P.R., Morrison J.H., and Gordon J.W. Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc. Natl Acad. Sci. USA 92 (1995) 689-693
    • (1995) Proc. Natl Acad. Sci. USA , vol.92 , pp. 689-693
    • Ripps, M.E.1    Huntley, G.W.2    Hof, P.R.3    Morrison, J.H.4    Gordon, J.W.5
  • 124
    • 0029854883 scopus 로고    scopus 로고
    • D90A heterozygosity in the SOD1 gene is associated with familial and apparently sporadic amyotrophic lateral sclerosis
    • Robberecht W., Aguirre T., Van Den Bosch L., Tilkin P., Cassiman J.J., and Matthijs G. D90A heterozygosity in the SOD1 gene is associated with familial and apparently sporadic amyotrophic lateral sclerosis. Neurology 47 (1996) 1336-1339
    • (1996) Neurology , vol.47 , pp. 1336-1339
    • Robberecht, W.1    Aguirre, T.2    Van Den Bosch, L.3    Tilkin, P.4    Cassiman, J.J.5    Matthijs, G.6
  • 125
    • 0030000608 scopus 로고    scopus 로고
    • Analysis of the KSP repeat of the neurofilament heavy subunit in familiar amyotrophic lateral sclerosis
    • Rooke K., Figlewicz D.A., Han F.Y., and Rouleau G.A. Analysis of the KSP repeat of the neurofilament heavy subunit in familiar amyotrophic lateral sclerosis. Neurology 46 (1996) 789-790
    • (1996) Neurology , vol.46 , pp. 789-790
    • Rooke, K.1    Figlewicz, D.A.2    Han, F.Y.3    Rouleau, G.A.4
  • 128
    • 0029030610 scopus 로고
    • Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis
    • Rothstein J.D., Van Kammen M., Levey A.I., Martin L.J., and Kuncl R.W. Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis. Ann. Neurol 38 (1995) 73-84
    • (1995) Ann. Neurol , vol.38 , pp. 73-84
    • Rothstein, J.D.1    Van Kammen, M.2    Levey, A.I.3    Martin, L.J.4    Kuncl, R.W.5
  • 133
    • 0033938202 scopus 로고    scopus 로고
    • Excitatory amino acid transporter 1 and 2 immunoreactivity in the spinal cord in amyotrophic lateral sclerosis
    • Sasaki S., Komori T., and Iwata M. Excitatory amino acid transporter 1 and 2 immunoreactivity in the spinal cord in amyotrophic lateral sclerosis. Acta. Neuropathol. (Berl) 100 (2000) 138-144
    • (2000) Acta. Neuropathol. (Berl) , vol.100 , pp. 138-144
    • Sasaki, S.1    Komori, T.2    Iwata, M.3
  • 134
    • 0029082389 scopus 로고
    • Oxidative damage to protein in sporadic motor neuron disease spinal cord
    • Shaw P.J., Ince P.G., Falkous G., and Mantle D. Oxidative damage to protein in sporadic motor neuron disease spinal cord. Ann. Neurol 38 (1995) 691-695
    • (1995) Ann. Neurol , vol.38 , pp. 691-695
    • Shaw, P.J.1    Ince, P.G.2    Falkous, G.3    Mantle, D.4
  • 136
    • 0035139109 scopus 로고    scopus 로고
    • Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases
    • Sherman M.Y., and Goldberg A.L. Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases. Neuron 29 (2001) 15-32
    • (2001) Neuron , vol.29 , pp. 15-32
    • Sherman, M.Y.1    Goldberg, A.L.2
  • 137
    • 0007731688 scopus 로고    scopus 로고
    • X-linked dominant locus for late-onset familial amyotrophic lateral sclerosis
    • Siddique N., Hong J.S., and Brooks B.J. X-linked dominant locus for late-onset familial amyotrophic lateral sclerosis. Am. J. Hum. Genet 63 (1998) A308
    • (1998) Am. J. Hum. Genet , vol.63
    • Siddique, N.1    Hong, J.S.2    Brooks, B.J.3
  • 139
    • 0032499788 scopus 로고    scopus 로고
    • Reexamination of the mechanism of hydroxyl radical adducts formed from the reaction between familial amyotrophic lateral sclerosis-associated Cu,Zn superoxide dismutase mutants and H2O2
    • Singh R.J., Karoui H., Gunther M.R., Beckman J.S., Mason R.P., and Kalyanaraman B. Reexamination of the mechanism of hydroxyl radical adducts formed from the reaction between familial amyotrophic lateral sclerosis-associated Cu,Zn superoxide dismutase mutants and H2O2. Proc. Natl Acad. Sci. USA 95 (1998) 6675-6680
    • (1998) Proc. Natl Acad. Sci. USA , vol.95 , pp. 6675-6680
    • Singh, R.J.1    Karoui, H.2    Gunther, M.R.3    Beckman, J.S.4    Mason, R.P.5    Kalyanaraman, B.6
  • 140
    • 0028924368 scopus 로고
    • Molecular approaches to amyotrophic lateral sclerosis
    • Smith R.G., and Appel S.H. Molecular approaches to amyotrophic lateral sclerosis. Annu. Rev. Med 46 (1995) 133-145
    • (1995) Annu. Rev. Med , vol.46 , pp. 133-145
    • Smith, R.G.1    Appel, S.H.2
  • 141
    • 0029666061 scopus 로고    scopus 로고
    • Apolipoprotein E epsilon 4 in bulbar-onset motor neuron disease
    • Smith R.G., Haverkamp L.J., Case S., Appel V., and Appel S.H. Apolipoprotein E epsilon 4 in bulbar-onset motor neuron disease. Lancet 348 (1996) 334-335
    • (1996) Lancet , vol.348 , pp. 334-335
    • Smith, R.G.1    Haverkamp, L.J.2    Case, S.3    Appel, V.4    Appel, S.H.5
  • 142
    • 0003374626 scopus 로고    scopus 로고
    • Tau protein pathology in neurodegenerative diseases
    • Spillantini M.G., and Goedert M. Tau protein pathology in neurodegenerative diseases. Trends Neurosci 21 (1998) 428-433
    • (1998) Trends Neurosci , vol.21 , pp. 428-433
    • Spillantini, M.G.1    Goedert, M.2
  • 143
    • 4644239349 scopus 로고    scopus 로고
    • VEGF, a modifier of motor neuron degeneration in SOD1 G93A mice, protects against motor neuron loss after spinal cord ischemia: Evidence for a vascular hypothesis
    • Program No. 602.11, Washington, DC: Society for Neuroscience, Online
    • Storkebaum, E, Lambrechts, D, Masafumi, M, Vanhoutte, G, Van der Linden, G, Collen, D, Baekelandt, V, Moons, L, Carmeliet, P (2003) VEGF, a modifier of motor neuron degeneration in SOD1 G93A mice, protects against motor neuron loss after spinal cord ischemia: evidence for a vascular hypothesis. Program No. 602.11. Abstract Viewer/Itinerary Planner. Washington, DC: Society for Neuroscience, 2003. Online.
    • (2003) Abstract Viewer/Itinerary Planner
    • Storkebaum, E.1    Lambrechts, D.2    Masafumi, M.3    Vanhoutte, G.4    Van der Linden, G.5    Collen, D.6    Baekelandt, V.7    Moons, L.8    Carmeliet, P.9
  • 144
    • 0026100188 scopus 로고
    • Familial amyotrophic lateral sclerosis, 1850-1989: a statistical analysis of the world literature
    • Strong M.J., Hudson A.J., and Alvord W.G. Familial amyotrophic lateral sclerosis, 1850-1989: a statistical analysis of the world literature. Can. J. Neurol. Sci 18 (1991) 45-58
    • (1991) Can. J. Neurol. Sci , vol.18 , pp. 45-58
    • Strong, M.J.1    Hudson, A.J.2    Alvord, W.G.3
  • 146
    • 0028021552 scopus 로고
    • A null mutation in the human CNTF gene is not causally related to neurological diseases
    • Takahashi R., Yokoji H., Misawa H., Hayashi M., Hu J., and Deguchi T. A null mutation in the human CNTF gene is not causally related to neurological diseases. Nat. Genet 7 (1994) 79-84
    • (1994) Nat. Genet , vol.7 , pp. 79-84
    • Takahashi, R.1    Yokoji, H.2    Misawa, H.3    Hayashi, M.4    Hu, J.5    Deguchi, T.6
  • 147
    • 0034203047 scopus 로고    scopus 로고
    • Demographic characteristics and prognosis in a Flemish amyotrophic lateral sclerosis population
    • Thijs V., Peeters E., Theys P., Matthijs G., and Robberecht W. Demographic characteristics and prognosis in a Flemish amyotrophic lateral sclerosis population. Acta. Neurol. Belg 100 (2000) 84-90
    • (2000) Acta. Neurol. Belg , vol.100 , pp. 84-90
    • Thijs, V.1    Peeters, E.2    Theys, P.3    Matthijs, G.4    Robberecht, W.5
  • 148
    • 0031816139 scopus 로고    scopus 로고
    • Distribution of MnSOD polymorphisms in sporadic ALS patients
    • Tomblyn M., Kasarskis E.J., Xu Y., and St Clair D.K. Distribution of MnSOD polymorphisms in sporadic ALS patients. J. Mol. Neurosci 10 (1998) 65-66
    • (1998) J. Mol. Neurosci , vol.10 , pp. 65-66
    • Tomblyn, M.1    Kasarskis, E.J.2    Xu, Y.3    St Clair, D.K.4
  • 149
    • 0035828146 scopus 로고    scopus 로고
    • Mutation screening of manganese superoxide dismutase in amyotrophic lateral sclerosis
    • Tomkins J., Banner S.J., McDermott C.J., and Shaw P.J. Mutation screening of manganese superoxide dismutase in amyotrophic lateral sclerosis. Neuroreport 12 (2001) 2319-2322
    • (2001) Neuroreport , vol.12 , pp. 2319-2322
    • Tomkins, J.1    Banner, S.J.2    McDermott, C.J.3    Shaw, P.J.4
  • 150
    • 0032427646 scopus 로고    scopus 로고
    • Novel insertion in the KSP region of the neurofilament heavy gene in amyotrophic lateral sclerosis (ALS)
    • Tomkins J., Usher P., Slade J.Y., Ince P.G., Curtis A., Bushby K., and Shaw P.J. Novel insertion in the KSP region of the neurofilament heavy gene in amyotrophic lateral sclerosis (ALS). Neuroreport 9 (1998) 3967-3970
    • (1998) Neuroreport , vol.9 , pp. 3967-3970
    • Tomkins, J.1    Usher, P.2    Slade, J.Y.3    Ince, P.G.4    Curtis, A.5    Bushby, K.6    Shaw, P.J.7
  • 151
    • 0033366461 scopus 로고    scopus 로고
    • SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter
    • Trotti D., Rolfs A., Danbolt N.C., Brown Jr. R.H., and Hediger M.A. SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter. Nat. Neurosci 2 (1999) 848
    • (1999) Nat. Neurosci , vol.2 , pp. 848
    • Trotti, D.1    Rolfs, A.2    Danbolt, N.C.3    Brown Jr., R.H.4    Hediger, M.A.5
  • 152
    • 0035808437 scopus 로고    scopus 로고
    • Amyotrophic lateral sclerosis-linked glutamate transporter mutant has impaired glutamate clearance capacity
    • Trotti D., Aoki M., Pasinelli P., Berger U.V., Danbolt N.C., Brown Jr. R.H., and Hediger M.A. Amyotrophic lateral sclerosis-linked glutamate transporter mutant has impaired glutamate clearance capacity. J. Biol. Chem 276 (2001) 576-582
    • (2001) J. Biol. Chem , vol.276 , pp. 576-582
    • Trotti, D.1    Aoki, M.2    Pasinelli, P.3    Berger, U.V.4    Danbolt, N.C.5    Brown Jr., R.H.6    Hediger, M.A.7
  • 153
    • 0037388067 scopus 로고    scopus 로고
    • Misfolded CuZnSOD and amyotrophic lateral sclerosis
    • Valentine J.S., and Hart P.J. Misfolded CuZnSOD and amyotrophic lateral sclerosis. Proc. Natl Acad. Sci. USA 100 (2003) 3617-3622
    • (2003) Proc. Natl Acad. Sci. USA , vol.100 , pp. 3617-3622
    • Valentine, J.S.1    Hart, P.J.2
  • 154
    • 0036713846 scopus 로고    scopus 로고
    • GluR2-dependent properties of AMPA receptors determine the selective vulnerability of motor neurons to excitotoxicity
    • Van Damme P., Van Den Bosch L., Van Houtte E., Callewaert G., and Robberecht W. GluR2-dependent properties of AMPA receptors determine the selective vulnerability of motor neurons to excitotoxicity. J. Neurophysiol 88 (2002) 1279-1287
    • (2002) J. Neurophysiol , vol.88 , pp. 1279-1287
    • Van Damme, P.1    Van Den Bosch, L.2    Van Houtte, E.3    Callewaert, G.4    Robberecht, W.5
  • 155
    • 0037986558 scopus 로고    scopus 로고
    • The AMPA receptor antagonist NBQX prolongs survival in a transgenic mouse model of amyotrophic lateral sclerosis
    • Van Damme P., Leyssen M., Callewaert G., Robberecht W., and Van Den Bosch L. The AMPA receptor antagonist NBQX prolongs survival in a transgenic mouse model of amyotrophic lateral sclerosis. Neurosci. Lett 343 (2003) 81-84
    • (2003) Neurosci. Lett , vol.343 , pp. 81-84
    • Van Damme, P.1    Leyssen, M.2    Callewaert, G.3    Robberecht, W.4    Van Den Bosch, L.5
  • 157
    • 0032779847 scopus 로고    scopus 로고
    • Manganese-containing superoxide dismutase signal sequence polymorphism associated with sporadic motor neuron disease
    • Van Landeghem G.F., Tabatabaie P., Beckman G., Beckman L., and Andersen P.M. Manganese-containing superoxide dismutase signal sequence polymorphism associated with sporadic motor neuron disease. Eur. J. Neurol 6 (1999) 639-644
    • (1999) Eur. J. Neurol , vol.6 , pp. 639-644
    • Van Landeghem, G.F.1    Tabatabaie, P.2    Beckman, G.3    Beckman, L.4    Andersen, P.M.5
  • 158
    • 77957036224 scopus 로고    scopus 로고
    • Unusual phenotype with the G93C mutation in SOD1 in familial ALS
    • Vanopdenbosch L., and Robberecht W. Unusual phenotype with the G93C mutation in SOD1 in familial ALS. Neurology 56 (2001) A445
    • (2001) Neurology , vol.56
    • Vanopdenbosch, L.1    Robberecht, W.2
  • 159
    • 0029970685 scopus 로고    scopus 로고
    • Sequence variants in human neurofilament proteins: absence of linkage to familial amyotrophic lateral sclerosis
    • Vechio J.D., Bruijn L.I., Xu Z., Brown Jr. R.H., and Cleveland D.W. Sequence variants in human neurofilament proteins: absence of linkage to familial amyotrophic lateral sclerosis. Ann. Neurol 40 (1996) 603-610
    • (1996) Ann. Neurol , vol.40 , pp. 603-610
    • Vechio, J.D.1    Bruijn, L.I.2    Xu, Z.3    Brown Jr., R.H.4    Cleveland, D.W.5
  • 161
    • 0025295206 scopus 로고
    • Autosomal dominant adult amyotrophic lateral sclerosis. A six generation Dutch family
    • Veltema A.N., Roos R.A., and Bruyn G.W. Autosomal dominant adult amyotrophic lateral sclerosis. A six generation Dutch family. J. Neurol. Sci 97 (1990) 93-115
    • (1990) J. Neurol. Sci , vol.97 , pp. 93-115
    • Veltema, A.N.1    Roos, R.A.2    Bruyn, G.W.3
  • 164
    • 0032727617 scopus 로고    scopus 로고
    • Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
    • Warrick J.M., Chan H.Y., Gray-Board G.L., Chai Y., Paulson H.L., and Bonini N.M. Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet 23 (1999) 425-428
    • (1999) Nat. Genet , vol.23 , pp. 425-428
    • Warrick, J.M.1    Chan, H.Y.2    Gray-Board, G.L.3    Chai, Y.4    Paulson, H.L.5    Bonini, N.M.6
  • 166
    • 0023759628 scopus 로고
    • Familial motor neuron disease: differing penetrance in large pedigrees
    • Williams D.B., Floate D.A., and Leicester J. Familial motor neuron disease: differing penetrance in large pedigrees. J. Neurol. Sci 86 (1988) 215-230
    • (1988) J. Neurol. Sci , vol.86 , pp. 215-230
    • Williams, D.B.1    Floate, D.A.2    Leicester, J.3
  • 167
    • 0033366384 scopus 로고    scopus 로고
    • Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons
    • Williamson T.L., and Cleveland D.W. Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nat. Neurosci 2 (1999) 50-56
    • (1999) Nat. Neurosci , vol.2 , pp. 50-56
    • Williamson, T.L.1    Cleveland, D.W.2
  • 168
    • 0032482976 scopus 로고    scopus 로고
    • Absence of neurofilaments reduces the selectivevulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant
    • Williamson T.L., Bruijn L.I., Zhu Q., Anderson K.L., Anderson S.D., Julien J.P., and Cleveland D.W. Absence of neurofilaments reduces the selectivevulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant. Proc. Natl Acad. Sci. USA 95 (1998) 9631-9636
    • (1998) Proc. Natl Acad. Sci. USA , vol.95 , pp. 9631-9636
    • Williamson, T.L.1    Bruijn, L.I.2    Zhu, Q.3    Anderson, K.L.4    Anderson, S.D.5    Julien, J.P.6    Cleveland, D.W.7
  • 169
    • 0029053881 scopus 로고
    • An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
    • Wong P.C., Pardo C.A., Borchelt D.R., Lee M.K., Copeland N.G., Jenkins N.A., Sisodia S.S., Cleveland D.W., and Price D.L. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14 (1995) 1105-1116
    • (1995) Neuron , vol.14 , pp. 1105-1116
    • Wong, P.C.1    Pardo, C.A.2    Borchelt, D.R.3    Lee, M.K.4    Copeland, N.G.5    Jenkins, N.A.6    Sisodia, S.S.7    Cleveland, D.W.8    Price, D.L.9
  • 170
    • 0031672857 scopus 로고    scopus 로고
    • The genetic and molecular mechanisms of motor neuron disease
    • Wong P.C., Rothstein J.D., and Price D.L. The genetic and molecular mechanisms of motor neuron disease. Curr. Opin. Neurobiol 8 (1998) 791-799
    • (1998) Curr. Opin. Neurobiol , vol.8 , pp. 791-799
    • Wong, P.C.1    Rothstein, J.D.2    Price, D.L.3
  • 171
    • 0027410516 scopus 로고
    • Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
    • Xu Z., Cork L.C., Griffin J.W., and Cleveland D.W. Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease. Cell 73 (1993) 23-33
    • (1993) Cell , vol.73 , pp. 23-33
    • Xu, Z.1    Cork, L.C.2    Griffin, J.W.3    Cleveland, D.W.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.