메뉴 건너뛰기




Volumn 12, Issue 5, 1999, Pages 581-588

Pathogenesis of amyotrophic lateral sclerosis: A critical review

Author keywords

[No Author keywords available]

Indexed keywords

CREATINE; LYSINE ACETYLSALICYLATE;

EID: 0033394926     PISSN: 13507540     EISSN: None     Source Type: Journal    
DOI: 10.1097/00019052-199910000-00012     Document Type: Review
Times cited : (28)

References (47)
  • 1
    • 0027401203 scopus 로고
    • Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    • 1 Rosen D, Siddique T, Patterson D, Figlewicz D, Sapp P, Hentati A, et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 1993; 362:59-62.
    • (1993) Nature , vol.362 , pp. 59-62
    • Rosen, D.1    Siddique, T.2    Patterson, D.3    Figlewicz, D.4    Sapp, P.5    Hentati, A.6
  • 2
    • 0032908774 scopus 로고    scopus 로고
    • Amyotrophic lateral sclerosis with mutations in superoxide dismutase: A putative mechanism of degeneration
    • 2 Morrison B, Morrison J. Amyotrophic lateral sclerosis with mutations in superoxide dismutase: a putative mechanism of degeneration. Brain Res Rev 1999; 29:121-135. This is a review of data from transgenic SOD I mice on the steps in the putative cascade that leads to neuronal death.
    • (1999) Brain Res Rev , vol.29 , pp. 121-135
    • Morrison, B.1    Morrison, J.2
  • 3
    • 0031015012 scopus 로고    scopus 로고
    • Abnormalities of cortical inhibitory neurons in amyotrophic lateral sclerosis
    • 3 Enterzari-Taher M, Eisen A, Stewart H, Nakajima M. Abnormalities of cortical inhibitory neurons in amyotrophic lateral sclerosis. Muscle Nerve 1997; 20:65-71.
    • (1997) Muscle Nerve , vol.20 , pp. 65-71
    • Enterzari-Taher, M.1    Eisen, A.2    Stewart, H.3    Nakajima, M.4
  • 4
    • 0033041668 scopus 로고    scopus 로고
    • Assessment of upperand lower motor neurons in Kennedy's disease: Implications for corticomotoneuronal PSTH studies
    • 4 Weber M, Eisen A. Assessment of upperand lower motor neurons in Kennedy's disease: implications for corticomotoneuronal PSTH studies. Muscle Nerve 1999; 22:299-306. This report contrasts abnormalities in upper motor neuron synaptic connections to lower motor neurons in individuals with ALS and Kennedy's disease, and supports a supraspinal pathologic mechanism in ALS.
    • (1999) Muscle Nerve , vol.22 , pp. 299-306
    • Weber, M.1    Eisen, A.2
  • 5
    • 0028142392 scopus 로고
    • El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis
    • 5 World Federation of Neurology Research Group on Neuromuscular Diseases. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 1994; 124 (Suppl):96-107.
    • (1994) J Neurol Sci , vol.124 , Issue.SUPPL. , pp. 96-107
  • 6
    • 0031895695 scopus 로고    scopus 로고
    • Toward earlier diagnosis of amyotrophic lateral sclerosis. Revised criteria
    • 6 Ross M, Miller R, Berchert L, Parry G, Barohn R, Armon C, et al. Toward earlier diagnosis of amyotrophic lateral sclerosis. Revised criteria. Neurology 1998; 50:768-772.
    • (1998) Neurology , vol.50 , pp. 768-772
    • Ross, M.1    Miller, R.2    Berchert, L.3    Parry, G.4    Barohn, R.5    Armon, C.6
  • 7
    • 0031814006 scopus 로고    scopus 로고
    • Limited corticospinal tract involvement in amyotrophic lateral sclerosis subjects with the A4V mutation in the copper/zinc superoxide dismutase gene
    • 7 Cudkowicz M, McKenna-Yasek D, Chen C, Hedley-Whyte E, Brown R. Limited corticospinal tract involvement in amyotrophic lateral sclerosis subjects with the A4V mutation in the copper/zinc superoxide dismutase gene. Ann Neurol 1998; 43:703-710. In families with the A4V SOD I mutation, not all affected members have upper motor neuron loss. This may require a revision of the clinical definition of ALS to make it more general.
    • (1998) Ann Neurol , vol.43 , pp. 703-710
    • Cudkowicz, M.1    McKenna-Yasek, D.2    Chen, C.3    Hedley-Whyte, E.4    Brown, R.5
  • 8
    • 9544236295 scopus 로고    scopus 로고
    • Autosomal recessive adult-onset amyotrophic lateral sclerosis associated with homozygosity for Asp90Ala CuZn-superoxide dismutase mutation. A clinical and genealogical study of 36 patients
    • 8 Andersen P, Forsgren L, Binzer M, Nilsson P, Ala-Hurula V, Keränen M-L, et al. Autosomal recessive adult-onset amyotrophic lateral sclerosis associated with homozygosity for Asp90Ala CuZn-superoxide dismutase mutation. A clinical and genealogical study of 36 patients. Brain 1996; 119: 1153-1172.
    • (1996) Brain , vol.119 , pp. 1153-1172
    • Andersen, P.1    Forsgren, L.2    Binzer, M.3    Nilsson, P.4    Ala-Hurula, V.5    Keränen, M.-L.6
  • 9
    • 7844227669 scopus 로고    scopus 로고
    • Recessive amyotrophic lateral sclerosis families with the D90a SOD1 mutation share a common founder: Evidence for a linked protective factor
    • 9 Al-Chalabi A, Andersen P, Chioza B, Shaw C, Sham P, Robberecht W, et al. Recessive amyotrophic lateral sclerosis families with the D90A SOD1 mutation share a common founder: evidence for a linked protective factor. Hum Mol Genet 1998; 13:2045-2050. Most SOD I mutations are autosomal dominant. The D90A mutation is an exception, and this report confirms that the D90A mutation can act in a dominant manner, but on one occasion it became recessive. They propose a tightly linked protective factor that reduces toxicity. More information about the protective factor could provide new insights into the mechanisms of SOD I pathology.
    • (1998) Hum Mol Genet , vol.13 , pp. 2045-2050
    • Al-Chalabi, A.1    Andersen, P.2    Chioza, B.3    Shaw, C.4    Sham, P.5    Robberecht, W.6
  • 11
    • 0000181477 scopus 로고    scopus 로고
    • Gender and presentation age in the ALS patient care database
    • 11 Cashman N, White C, Anderson F, Group ACS. Gender and presentation age in the ALS Patient Care Database [abstract]. Neurology 1999; 52:A116. Although only in abstract form, it represents an epidemiologic approach to investigating the pathogenesis of ALS. A greater incidence of ALS in older, presumably postmenopausal women suggests a protective effect of estrogen in premenopausal women.
    • (1999) Neurology , vol.52
    • Cashman, N.1    White, C.2    Anderson, F.3
  • 12
    • 0032079517 scopus 로고    scopus 로고
    • Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expression a mutant SOD1
    • 12 Kong J, Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expression a mutant SOD1. J Neurosci 1998; 18:3241-3250. By following mutant SOD I transgenic mice according to the stage of the disease and not age of the mice, mitochondrial degeneration was an early change. These data open abnormal energy as a pathogenic factor.
    • (1998) J Neurosci , vol.18 , pp. 3241-3250
    • Kong, J.1    Xu, Z.2
  • 13
    • 0029847118 scopus 로고    scopus 로고
    • Excitotoxicity hypothesis
    • 13 Rothstein J. Excitotoxicity hypothesis. Neurology 1996; 47 (Suppl 2):S19-S26.
    • (1996) Neurology , vol.47 , Issue.SUPPL. 2
    • Rothstein, J.1
  • 14
    • 0032032013 scopus 로고    scopus 로고
    • Aberrant RNA processing in a neurodegenerative disease: The cause of absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis
    • 14 Lin C-LG, Bristol L, Jin L, Dykes-Hoberg M, Crawford T, Clawson L, et al. Aberrant RNA processing in a neurodegenerative disease: the cause of absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis. Neuron 1998; 20:589-602.
    • (1998) Neuron , vol.20 , pp. 589-602
    • Lin, C.-L.G.1    Bristol, L.2    Jin, L.3    Dykes-Hoberg, M.4    Crawford, T.5    Clawson, L.6
  • 15
    • 0031957298 scopus 로고    scopus 로고
    • Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis
    • 15 Aoki M, Lin C, Rothstein J, Geller B, Hosler B, Munsat T, et al. Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis. Ann Neurol 1998; 43:645-653. Mutations in the EAAT2 gene are unlikely to account for the presence of variant messenger RNA transcripts observed in 60% of sporadic ALS patients.
    • (1998) Ann Neurol , vol.43 , pp. 645-653
    • Aoki, M.1    Lin, C.2    Rothstein, J.3    Geller, B.4    Hosler, B.5    Munsat, T.6
  • 16
    • 0032894660 scopus 로고    scopus 로고
    • Decreased striatal dopamine-receptor binding in sporadic ALS: Glutamate hyperactivity?
    • 16 Vogels O, Oyen W, van Engelen B, Padberg G, Horsink M. Decreased striatal dopamine-receptor binding in sporadic ALS: glutamate hyperactivity? Neurology 1999; 52:1275-1277.
    • (1999) Neurology , vol.52 , pp. 1275-1277
    • Vogels, O.1    Oyen, W.2    Van Engelen, B.3    Padberg, G.4    Horsink, M.5
  • 17
    • 0028085696 scopus 로고
    • The role of intracellular free calcium in motor neuron disease
    • 17 Krieger C, Jones K, Kim S, Eisen A. The role of intracellular free calcium in motor neuron disease. J Neurol Sci 1994; 124 (Suppl) :27-31.
    • (1994) J Neurol Sci , vol.124 , Issue.SUPPL. , pp. 27-31
    • Krieger, C.1    Jones, K.2    Kim, S.3    Eisen, A.4
  • 18
    • 0032402093 scopus 로고    scopus 로고
    • Glutamate potentiates the toxicity of mutant Cu/Zu-superoxide dismutase in motor neurons by post-synaptic calcium-dependent mechanisms
    • 18 Roy J, Minotti S, Dong L, Figlewicz D, Durham H. Glutamate potentiates the toxicity of mutant Cu/Zu-superoxide dismutase in motor neurons by post-synaptic calcium-dependent mechanisms. J Neurosci 1998; 18:9673-9684. Data from a mutant SOD I cell culture model showing calcium influx through non-NMDA receptors and to a lesser degree through calcium channels.
    • (1998) J Neurosci , vol.18 , pp. 9673-9684
    • Roy, J.1    Minotti, S.2    Dong, L.3    Figlewicz, D.4    Durham, H.5
  • 19
    • 0027946813 scopus 로고
    • The role of calcium-binding proteins in selective motorneruon vulnerability in amyotrophic lateral sclerosis
    • 19 Alexianu M, Ho B-K, Mohamed A, La Bella V, Smith R, Appel S. The role of calcium-binding proteins in selective motorneruon vulnerability in amyotrophic lateral sclerosis. Ann Neurol 1994; 36:846-858.
    • (1994) Ann Neurol , vol.36 , pp. 846-858
    • Alexianu, M.1    Ho, B.-K.2    Mohamed, A.3    La Bella, V.4    Smith, R.5    Appel, S.6
  • 20
    • 0033547879 scopus 로고    scopus 로고
    • S-100β is up regulated in astrocytes and motor neurons in the spinal cord of patients with amyotrophic lateral sclerosis
    • 20 Migheli A, Cordera S, Bendotti C, Atzori C, Piva R, Schiffer D. S-100β is up regulated in astrocytes and motor neurons in the spinal cord of patients with amyotrophic lateral sclerosis. Neurosci Lett 1999; 261 :25-28. This report describes upregulation of a calcium binding protein in astrocytes and neurons in postmortem ALS spinal cords compared with non-neurologic control individuals. Upregulation could represent a possible defense mechanism to neuronal injury.
    • (1999) Neurosci Lett , vol.261 , pp. 25-28
    • Migheli, A.1    Cordera, S.2    Bendotti, C.3    Atzori, C.4    Piva, R.5    Schiffer, D.6
  • 21
    • 0031768025 scopus 로고    scopus 로고
    • Enhanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis
    • 21 Liu R, Althaus J, Ellerbrock B, Becker D, Gurney M. Enhanced oxygen radical production in a transgenic mouse model of familial amyotrophic lateral sclerosis. Ann Neurol 1998; 44:763-770.
    • (1998) Ann Neurol , vol.44 , pp. 763-770
    • Liu, R.1    Althaus, J.2    Ellerbrock, B.3    Becker, D.4    Gurney, M.5
  • 22
    • 0032904412 scopus 로고    scopus 로고
    • An evaluation of the role of mitochondria in neurodegenerative diseases: Mitochondrial mutations and oxidative pathology, protective nuclear responses, and cell death in neurodegeneration
    • 22 Cassarino D, Bennett J. An evaluation of the role of mitochondria in neurodegenerative diseases: mitochondrial mutations and oxidative pathology, protective nuclear responses, and cell death in neurodegeneration. Brain Res Rev 1999; 29:1-25. This is a review of data on the role of mitochondria in neurodegenerative disorders, including ALS.
    • (1999) Brain Res Rev , vol.29 , pp. 1-25
    • Cassarino, D.1    Bennett, J.2
  • 23
    • 0029053881 scopus 로고
    • An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
    • 23 Wong P, Pardo C, Borchelt D, Lee M, Copeland N, Jenkins N, et al. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 1995; 14:1105-1116.
    • (1995) Neuron , vol.14 , pp. 1105-1116
    • Wong, P.1    Pardo, C.2    Borchelt, D.3    Lee, M.4    Copeland, N.5    Jenkins, N.6
  • 24
    • 0030851761 scopus 로고    scopus 로고
    • Increased 3-nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis
    • 24 Beal M, Ferrante R, Browne S, Matthews R, Kowall N, Brown R. Increased 3-nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis. Ann Neurol 1997; 42:646-654.
    • (1997) Ann Neurol , vol.42 , pp. 646-654
    • Beal, M.1    Ferrante, R.2    Browne, S.3    Matthews, R.4    Kowall, N.5    Brown, R.6
  • 25
    • 0031724093 scopus 로고    scopus 로고
    • Expression of nitric oxide synthase in the spinal cord in amyotrophic lateral sclerosis
    • 25 Phul R, Smith M, Shaw P, Ince P. Expression of nitric oxide synthase in the spinal cord in amyotrophic lateral sclerosis. J Neurol Sci 1998; 160 (Suppl. 1):S87-S91.
    • (1998) J Neurol Sci , vol.160 , Issue.SUPPL. 1
    • Phul, R.1    Smith, M.2    Shaw, P.3    Ince, P.4
  • 26
    • 0032499878 scopus 로고    scopus 로고
    • Nitration of the low molecular weight neurofilament is equivalent in sporadic amyotrophic lateral sclerosis and control cervical spinal cord
    • 26 Strong M, Sopper M, Crow J, Strang W, Beckman J. Nitration of the low molecular weight neurofilament is equivalent in sporadic amyotrophic lateral sclerosis and control cervical spinal cord. Biochem Biophys Res Commun 1998; 248: 157-164. This is a study of postmortem spinal cord tissue from sporadic ALS and non-ALS individuals in which no differences were found in nitration of small neurofilament subunits. This is evidence against nitration as a major pathophysiologic mechanism.
    • (1998) Biochem Biophys Res Commun , vol.248 , pp. 157-164
    • Strong, M.1    Sopper, M.2    Crow, J.3    Strang, W.4    Beckman, J.5
  • 27
    • 0033064535 scopus 로고    scopus 로고
    • Administration of nitric oxide synthase inhibitors does not alter disease course of amyotrophic lateral sclerosis SOD1 mutant transgenic mice
    • 27 Upton-Rice M, Cudkowicz M, Mathew R, Reif D, Brown R. Administration of nitric oxide synthase inhibitors does not alter disease course of amyotrophic lateral sclerosis SOD1 mutant transgenic mice. Ann Neurol 1999; 45:413-414.
    • (1999) Ann Neurol , vol.45 , pp. 413-414
    • Upton-Rice, M.1    Cudkowicz, M.2    Mathew, R.3    Reif, D.4    Brown, R.5
  • 28
    • 0031795392 scopus 로고    scopus 로고
    • Mechanism of selective motor neuronal death after exposure of spinal cord to glutamate: Involvement of glutamate-induced nitric oxide in motor neuron toxicity and nonmotor neuron protection
    • 28 Urushitani M, Shimohama S, Kihara T, Sawada H, Akaike A, Ibi M, et al. Mechanism of selective motor neuronal death after exposure of spinal cord to glutamate: involvement of glutamate-induced nitric oxide in motor neuron toxicity and nonmotor neuron protection. Ann Neurol 1998; 44:796-807.
    • (1998) Ann Neurol , vol.44 , pp. 796-807
    • Urushitani, M.1    Shimohama, S.2    Kihara, T.3    Sawada, H.4    Akaike, A.5    Ibi, M.6
  • 29
    • 0008910729 scopus 로고    scopus 로고
    • Reply
    • 29 Beal M. Reply. Ann Neurol 1999; 45:414.
    • (1999) Ann Neurol , vol.45 , pp. 414
    • Beal, M.1
  • 30
    • 0031683605 scopus 로고    scopus 로고
    • Transgenic mice in the study of ALS: The role of neurofilaments
    • 30 Julien J-P, Couillard-Després, Meler J. Transgenic mice in the study of ALS: the role of neurofilaments. Brain Pathol 1998; 8:759-769. This is a review of data on neurofilaments in transgenic SOD I mouse models.
    • (1998) Brain Pathol , vol.8 , pp. 759-769
    • Julien, J.-P.1    Couillard-Després, M.J.2
  • 31
    • 0032926368 scopus 로고    scopus 로고
    • Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis
    • 31 Al-Chalabi A, Andersen P, Nilsson P, Chioza B, Andersson J, Russ C, et al. Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis. Hum Mol Genet 1999; 8: 157-64. Genetic abnormalities beyond SOD I mutations have been challenging. Al-Chalabi et al. described finding four mutations in the heavy neurofilament subunit in both sporadic and familial patients and control individuals. Interestingly, motor neuron disease was present only when the mutant allele was paired with the normal allele coding for the longer of the heavy subunits. Although these mutations are rare, they open the door to pathogenetic mechanisms involving this portion of neurofilaments.
    • (1999) Hum Mol Genet , vol.8 , pp. 157-164
    • Al-Chalabi, A.1    Andersen, P.2    Nilsson, P.3    Chioza, B.4    Andersson, J.5    Russ, C.6
  • 32
    • 0032482976 scopus 로고    scopus 로고
    • 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
    • 32 Williamson T, Bruijn L, Zhu Q, Anderson K, Anderson S, Julien J, et al. 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 1998; 95:9631-9636. SOD I transgenic mice that also have a disrupted neurofilament light chain gene and hence do not assemble neurofilaments were shown to live longer that SOD I mutant mice with normal neurofilaments. This indicates that neurofilaments are not essential for SOD I-mediated disease.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 9631-9636
    • Williamson, T.1    Bruijn, L.2    Zhu, Q.3    Anderson, K.4    Anderson, S.5    Julien, J.6
  • 33
    • 0032483016 scopus 로고    scopus 로고
    • Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
    • 33 Couillard-Després S, Zhu Q, Wong P, Price D, Cleveland D, Julien J. Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase. Proc Natl Acad Sci USA 1998; 95:9626-9630. Overexpression of the heavy neurofilament subunit in transgenic SOD I mice is protective.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 9626-9630
    • Couillard-Després, S.1    Zhu, Q.2    Wong, P.3    Price, D.4    Cleveland, D.5    Julien, J.6
  • 34
    • 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
    • 34 Williamson T, Cleveland D. Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nature Neurosci 1999; 2:50-56. Rates of axonal transport, especially tubulin, is slow, and maybe herald early changes in SOD I mutant mice.
    • (1999) Nature Neurosci , vol.2 , pp. 50-56
    • Williamson, T.1    Cleveland, D.2
  • 35
    • 0031876839 scopus 로고    scopus 로고
    • Advanced glycation endproducts in neurofilament conglomeration of motorneurons in familial and sporadic amytorphic lateral sclerosis
    • 35 Chou S, Wang H, Taniguchi A, Bucala R. Advanced glycation endproducts in neurofilament conglomeration of motorneurons in familial and sporadic amytorphic lateral sclerosis. Mol Med 1998; 4:324-332.
    • (1998) Mol Med , vol.4 , pp. 324-332
    • Chou, S.1    Wang, H.2    Taniguchi, A.3    Bucala, R.4
  • 36
    • 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
    • 36 Bruening W, Roy J, Giasson B, Figlewicz D, Mushynski W, Durham H. Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis. J Neurochem 1999; 72:693-699.
    • (1999) J Neurochem , vol.72 , pp. 693-699
    • Bruening, W.1    Roy, J.2    Giasson, B.3    Figlewicz, D.4    Mushynski, W.5    Durham, H.6
  • 37
    • 0032901111 scopus 로고    scopus 로고
    • The copper chaperone CCS is abundant in neurons and astrocytes in human and rodent brain
    • 37 Rothstein J, Dykes-Hoberg M, Corson L, Becker M, Cleveland D, Price D, et al. The copper chaperone CCS is abundant in neurons and astrocytes in human and rodent brain. J Neurochem 1999; 72:422-429. A copper chaperone protein specific for SOD I enzyme in rat and human central nervous system colocalizes in neurons that degenerate in ALS. These data open a new pathologic pathway for ALS.
    • (1999) J Neurochem , vol.72 , pp. 422-429
    • Rothstein, J.1    Dykes-Hoberg, M.2    Corson, L.3    Becker, M.4    Cleveland, D.5    Price, D.6
  • 38
    • 0032508609 scopus 로고    scopus 로고
    • The copper chaperone CCS directly interacts with Copper/Zinc superoxide dismutase
    • 38 Casareno R, Waggoner D, Gitlin J. The copper chaperone CCS directly interacts with Copper/Zinc superoxide dismutase. J Biol Chem 1998; 273:23625-23628. Report of specific interactions between a specific copper chaperone protein and both wild-type and a common SOD I mutation. These data open a new pathologic pathway for ALS.
    • (1998) J Biol Chem , vol.273 , pp. 23625-23628
    • Casareno, R.1    Waggoner, D.2    Gitlin, J.3
  • 39
    • 0032568546 scopus 로고    scopus 로고
    • Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants
    • 39 Corson L, Strain J, Culotta V, Cleveland D. Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants. Proc Natl Acad Sci USA 1998; 95:6361-6366.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 6361-6366
    • Corson, L.1    Strain, J.2    Culotta, V.3    Cleveland, D.4
  • 40
    • 0032430185 scopus 로고    scopus 로고
    • Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase
    • 40 Pasinelli P, Borchelt D, Housewert M, Cleveland D, Brown R. Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase. Proc Natl Acad Sci USA 1998; 95: 15763-15768.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 15763-15768
    • Pasinelli, P.1    Borchelt, D.2    Housewert, M.3    Cleveland, D.4    Brown, R.5
  • 41
    • 0032896327 scopus 로고    scopus 로고
    • Neuronal death in amyotrophic lateral sclerosis is apoptosis: Possible contribution of a programmed cell death mechanism
    • 41 Martin L. Neuronal death in amyotrophic lateral sclerosis is apoptosis: possible contribution of a programmed cell death mechanism. J Neuropathol Exp Neurol 1999;58:459-471. This is the first clear report from ALS postmortem spinal cord that supports apoptotic cell death in ALS.
    • (1999) J Neuropathol Exp Neurol , vol.58 , pp. 459-471
    • Martin, L.1
  • 42
    • 0033051815 scopus 로고    scopus 로고
    • Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
    • 42 Klivenyi P, Ferrante R, Matthews R, Bogdanov M, Klein A, Andreassen O, et al. Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis. Nature Med 1999; 5:347-350. This report raised interest among ALS patients for a simple and available treatment for the disease. However, this is a preliminary report that raises interesting questions, but needs to be replicated.
    • (1999) Nature Med , vol.5 , pp. 347-350
    • Klivenyi, P.1    Ferrante, R.2    Matthews, R.3    Bogdanov, M.4    Klein, A.5    Andreassen, O.6
  • 43
    • 0030050727 scopus 로고    scopus 로고
    • Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis
    • 43 Gurney M, Cutting F, Zhai P, Doble A, Taylor C, Andrus P, et al. Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis. Ann Neurol 1996; 39: 147-157.
    • (1996) Ann Neurol , vol.39 , pp. 147-157
    • Gurney, M.1    Cutting, F.2    Zhai, P.3    Doble, A.4    Taylor, C.5    Andrus, P.6
  • 44
    • 0033541016 scopus 로고    scopus 로고
    • Creatine monohydrate increases strength in patients with neuromuscular disease
    • 44 Tarnopolsky M, Martin J. Creatine monohydrate increases strength in patients with neuromuscular disease. Neurology 1999; 52:854-857.
    • (1999) Neurology , vol.52 , pp. 854-857
    • Tarnopolsky, M.1    Martin, J.2
  • 45
    • 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
    • 45 Barnéoud P, 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 1999; 155:243-251.
    • (1999) Exp Neurol , vol.155 , pp. 243-251
    • Barnéoud, P.1    Curet, O.2
  • 46
    • 0031672857 scopus 로고    scopus 로고
    • The genetic and molecular mechanisms of motor neuron disease
    • 46 Wong P, Rothstein J, Price D. The genetic and molecular mechanisms of motor neuron disease. Curr Opin Neurobiol 1998; 8:791-799. This is a review of genetic aspects of ALS, including mutant SOD I transgenic mice, and spinal muscular atrophy. It also includes a review of excitotoxicity and the role of copper.
    • (1998) Curr Opin Neurobiol , vol.8 , pp. 791-799
    • Wong, P.1    Rothstein, J.2    Price, D.3
  • 47
    • 3543045520 scopus 로고    scopus 로고
    • Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis
    • 47 Morrison B, Morrison J, Gordon J. Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis. J Exp Zool 1998; 28:32-47. This is a review of data on neurofilaments in transgenic SOD I mouse models.
    • (1998) J Exp Zool , vol.28 , pp. 32-47
    • Morrison, B.1    Morrison, J.2    Gordon, J.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.