메뉴 건너뛰기




Volumn 3, Issue 4, 2013, Pages 431-457

Characterization of early pathogenesis in the SOD1G93A mouse model of ALS: Part II, results and discussion

Author keywords

Axons; Cytoplasmic vacuoles; Glia; Mega mitochondria; Mitochondria; Motoneurons; Motor function; NMJs

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; MEMBRANE PROTEIN; PROTEIN P14; PROTEIN P30; PROTEIN P60; PROTEIN P7; PROTEIN P75; UNCLASSIFIED DRUG;

EID: 84893493940     PISSN: None     EISSN: 21623279     Source Type: Journal    
DOI: 10.1002/brb3.142     Document Type: Article
Times cited : (133)

References (132)
  • 1
    • 0036326289 scopus 로고    scopus 로고
    • Detection of preclinical motor neurone loss in SOD1 mutation carriers using motor unit number estimation
    • Aggarwal, A., and G. Nicholson. 2002. Detection of preclinical motor neurone loss in SOD1 mutation carriers using motor unit number estimation. J. Neurol. Neursurg. Psych. 73:199-201.
    • (2002) J. Neurol. Neursurg. Psych. , vol.73 , pp. 199-201
    • Aggarwal, A.1    Nicholson, G.2
  • 3
    • 36248986621 scopus 로고    scopus 로고
    • Postnatal electrical and morphological abnormalities in lumbar motoneurons from transgenic mouse models of amyotrophic lateral sclerosis
    • Amendola, J., J. P. Gueritaud, B. L. d'Incamps, C. Bories, S. Liabeuf, C. Allene, et al. 2007. Postnatal electrical and morphological abnormalities in lumbar motoneurons from transgenic mouse models of amyotrophic lateral sclerosis. Arch. Ital. Biol. 145:311-323.
    • (2007) Arch. Ital. Biol. , vol.145 , pp. 311-323
    • Amendola, J.1    Gueritaud, J.P.2    d'Incamps, B.L.3    Bories, C.4    Liabeuf, S.5    Allene, C.6
  • 4
    • 0016156347 scopus 로고
    • Lower motor neuron degeneration associated with type C RNA virus infection in mice: neuropathological features
    • Andrews, J. M., and M. B. Gardner. 1974. Lower motor neuron degeneration associated with type C RNA virus infection in mice: neuropathological features. J. Neuropathol. Exp. Neurol. 33:285-307.
    • (1974) J. Neuropathol. Exp. Neurol. , vol.33 , pp. 285-307
    • Andrews, J.M.1    Gardner, M.B.2
  • 6
    • 0035886428 scopus 로고    scopus 로고
    • Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity
    • Bendotti, C., N. Calvaresi, L. Chiveri, A. Prelle, M. Moggio, M. Braga, et al. 2001. Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity. J. Neurol. Sci. 191:25-33.
    • (2001) J. Neurol. Sci. , vol.191 , pp. 25-33
    • Bendotti, C.1    Calvaresi, N.2    Chiveri, L.3    Prelle, A.4    Moggio, M.5    Braga, M.6
  • 7
    • 35649017117 scopus 로고    scopus 로고
    • Dynamic remodelling of synapses can occur in the absence of the parent cell body
    • Bettini, N. L., T. S. Moores, B. Baxter, J. Deuchars, and S. H. Parson. 2007. Dynamic remodelling of synapses can occur in the absence of the parent cell body. BMC Neurosci. 8:79.
    • (2007) BMC Neurosci. , vol.8 , pp. 79
    • Bettini, N.L.1    Moores, T.S.2    Baxter, B.3    Deuchars, J.4    Parson, S.H.5
  • 9
    • 35648971133 scopus 로고    scopus 로고
    • Early diagnosis of ALS: the search for signs of denervation in clinically normal muscles
    • Blijham, P. J., H. J. Schelhaas, H. J. Ter Laak, B. G. van Engelen, and M. J. Zwarts. 2007. Early diagnosis of ALS: the search for signs of denervation in clinically normal muscles. J. Neurol. Sci. 263:154-157.
    • (2007) J. Neurol. Sci. , vol.263 , pp. 154-157
    • Blijham, P.J.1    Schelhaas, H.J.2    Ter Laak, H.J.3    van Engelen, B.G.4    Zwarts, M.J.5
  • 10
    • 84883847864 scopus 로고
    • An electron-microscopic study of the monkey spinal cord. I. Fine structure of normal motor column. II. Effects of retrograde chromatolysis. III. Cytologic effects of mild and virulent poliovirus infection
    • Bodian, D. 1964. An electron-microscopic study of the monkey spinal cord. I. Fine structure of normal motor column. II. Effects of retrograde chromatolysis. III. Cytologic effects of mild and virulent poliovirus infection. Bull. Johns Hopkins Hosp. 114:13-119.
    • (1964) Bull. Johns Hopkins Hosp. , vol.114 , pp. 13-119
    • Bodian, D.1
  • 11
    • 33749056809 scopus 로고    scopus 로고
    • ALS: a disease of motor neurons and their nonneuronal neighbors
    • Boillée, S., C. Vande Velde, and D. W. Cleveland. 2006. ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron 52:39-59.
    • (2006) Neuron , vol.52 , pp. 39-59
    • Boillée, S.1    Vande Velde, C.2    Cleveland, D.W.3
  • 12
    • 33847270640 scopus 로고    scopus 로고
    • Survival and death of mature avian motoneurons in organotypic slice culture: trophic requirements for survival and different types of degeneration
    • Brunet, N., O. Tarabal, M. Portero-Otín, R. W. Oppenheim, J. E. Esquerda, and J. Calderó. 2007. Survival and death of mature avian motoneurons in organotypic slice culture: trophic requirements for survival and different types of degeneration. J. Comp. Neurol. 501:669-690.
    • (2007) J. Comp. Neurol. , vol.501 , pp. 669-690
    • Brunet, N.1    Tarabal, O.2    Portero-Otín, M.3    Oppenheim, R.W.4    Esquerda, J.E.5    Calderó, J.6
  • 13
    • 69449094619 scopus 로고    scopus 로고
    • Excitotoxic motoneuron degeneration induced by glutamate receptor agonists and mitochondrial toxins in organotypic cultures of chick embryo spinal cord
    • Brunet, N., O. Tarabal, J. E. Esquerda, and J. Calderó. 2009. Excitotoxic motoneuron degeneration induced by glutamate receptor agonists and mitochondrial toxins in organotypic cultures of chick embryo spinal cord. J. Comp. Neurol. 516:277-290.
    • (2009) J. Comp. Neurol. , vol.516 , pp. 277-290
    • Brunet, N.1    Tarabal, O.2    Esquerda, J.E.3    Calderó, J.4
  • 14
    • 84856220351 scopus 로고    scopus 로고
    • Mouse transgenic lines that selectively label type I, type IIA and types IIX+B skeletal muscle fibers
    • Chakkalakal, J. V., S. Kuang, M. Buffelli, J. W. Lichtman, and J. R. Sanes. 2012. Mouse transgenic lines that selectively label type I, type IIA and types IIX+B skeletal muscle fibers. Genesis 50:50-58.
    • (2012) Genesis , vol.50 , pp. 50-58
    • Chakkalakal, J.V.1    Kuang, S.2    Buffelli, M.3    Lichtman, J.W.4    Sanes, J.R.5
  • 15
    • 0015320027 scopus 로고
    • Electron microscopic histochemical study on the localization and distribution of mercury in the nervous system after mercury intoxication
    • Chang, L. W., and H. A. Hartmann. 1972. Electron microscopic histochemical study on the localization and distribution of mercury in the nervous system after mercury intoxication. Exp. Neurol. 35:122-137.
    • (1972) Exp. Neurol. , vol.35 , pp. 122-137
    • Chang, L.W.1    Hartmann, H.A.2
  • 16
    • 11844296730 scopus 로고    scopus 로고
    • Bax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion disease
    • Chiesa, R., P. Piccardo, S. Dossena, L. Nowoslawski, K. A. Roth, and Ghetti. 2005. Bax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion disease. Proc. Natl. Acad. Sci. USA 102:238-243.
    • (2005) Proc. Natl. Acad. Sci. USA , vol.102 , pp. 238-243
    • Chiesa, R.1    Piccardo, P.2    Dossena, S.3    Nowoslawski, L.4    Roth, K.A.5    Ghetti6
  • 17
    • 0029096114 scopus 로고
    • Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis
    • Chiu, A. Y., P. Zhai, M. C. Dal Canto, T. M. Peters, Y. W. Kwon, S. M. Prattis, et al. 1995. Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Mol. Cell. Neurosci. 6:349-362.
    • (1995) Mol. Cell. Neurosci. , vol.6 , pp. 349-362
    • Chiu, A.Y.1    Zhai, P.2    Dal Canto, M.C.3    Peters, T.M.4    Kwon, Y.W.5    Prattis, S.M.6
  • 18
    • 0029185514 scopus 로고
    • Neuron death in vertebrate development: in vivo methods
    • Clarke, P. G., and R. W. Oppenheim. 1995. Neuron death in vertebrate development: in vivo methods. Methods Cell Biol. 46:277-321.
    • (1995) Methods Cell Biol. , vol.46 , pp. 277-321
    • Clarke, P.G.1    Oppenheim, R.W.2
  • 19
    • 0036774951 scopus 로고    scopus 로고
    • Axon pathology in neurological disease: a neglected therapeutic target
    • Coleman, M. P., and V. H. Perry. 2002. Axon pathology in neurological disease: a neglected therapeutic target. Trends Neurosci. 25:532-537.
    • (2002) Trends Neurosci. , vol.25 , pp. 532-537
    • Coleman, M.P.1    Perry, V.H.2
  • 21
    • 0014657859 scopus 로고
    • Observations on the ultrastructure of the initial motor axon segment and dorsal root boutons on the motoneurons in the lumbosacral spinal cord of the cat during postnatal development
    • Conradi, S., and S. Skoglund. 1969. Observations on the ultrastructure of the initial motor axon segment and dorsal root boutons on the motoneurons in the lumbosacral spinal cord of the cat during postnatal development. Acta Physiol. Scand. Suppl. 333:53-76.
    • (1969) Acta Physiol. Scand. Suppl. , vol.333 , pp. 53-76
    • Conradi, S.1    Skoglund, S.2
  • 22
    • 33846389267 scopus 로고    scopus 로고
    • Pro-NGF secreted by astrocytes promotes motor neuron cell death
    • Domeniconi, M., B. L. Hempstead, and M. V. Chao. 2007. Pro-NGF secreted by astrocytes promotes motor neuron cell death. Mol. Cell. Neurosci. 34:271-279.
    • (2007) Mol. Cell. Neurosci. , vol.34 , pp. 271-279
    • Domeniconi, M.1    Hempstead, B.L.2    Chao, M.V.3
  • 23
    • 0028802665 scopus 로고
    • Amyotrophic lateral sclerosis
    • Eisen, A. 1995. Amyotrophic lateral sclerosis. Intern. Med. 34:824-832.
    • (1995) Intern. Med. , vol.34 , pp. 824-832
    • Eisen, A.1
  • 25
    • 0344950387 scopus 로고    scopus 로고
    • Inhibiting axon degeneration and synapse loss attenuates apoptosis and disease progression in a mouse model of motoneuron disease
    • Ferri, A., J. R. Sanes, M. P. Coleman, J. M. Cunningham, and A. C. Kato. 2003. Inhibiting axon degeneration and synapse loss attenuates apoptosis and disease progression in a mouse model of motoneuron disease. Curr. Biol. 13:669-673.
    • (2003) Curr. Biol. , vol.13 , pp. 669-673
    • Ferri, A.1    Sanes, J.R.2    Coleman, M.P.3    Cunningham, J.M.4    Kato, A.C.5
  • 27
    • 71149086915 scopus 로고    scopus 로고
    • A new class of spinal interneurons: the origin and function of C boutons is solved
    • Frank, E. 2009. A new class of spinal interneurons: the origin and function of C boutons is solved. Neuron 64:593-595.
    • (2009) Neuron , vol.64 , pp. 593-595
    • Frank, E.1
  • 28
    • 0034175513 scopus 로고    scopus 로고
    • Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases
    • Frey, D., C. Schneider, L. Xu, J. Borg, W. Spooren, and P. Caroni. 2000. Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases. J. Neurosci. 20:2534-2542.
    • (2000) J. Neurosci. , vol.20 , pp. 2534-2542
    • Frey, D.1    Schneider, C.2    Xu, L.3    Borg, J.4    Spooren, W.5    Caroni, P.6
  • 29
    • 0015715763 scopus 로고
    • A spontaneous lower motor neuron disease apparently caused by indigenous type-C RNA virus in wild mice
    • Gardner, M. B., B. E. Henderson, J. E. Officer, R. W. Rongey, J. C. Parker, C. Oliver, et al. 1973. A spontaneous lower motor neuron disease apparently caused by indigenous type-C RNA virus in wild mice. J. Natl. Cancer Inst. 51:1243-1254.
    • (1973) J. Natl. Cancer Inst. , vol.51 , pp. 1243-1254
    • Gardner, M.B.1    Henderson, B.E.2    Officer, J.E.3    Rongey, R.W.4    Parker, J.C.5    Oliver, C.6
  • 30
    • 84865824804 scopus 로고    scopus 로고
    • Early functional deficit and microglial disturbances in a mouse model of amyotrophic lateral sclerosis
    • doi: 10.1371/journal.pone.0036000.
    • Gerber, Y. N., J. C. Sabourin, M. Rabano, M. D. Vivanco, and F. E. Perrin. 2012. Early functional deficit and microglial disturbances in a mouse model of amyotrophic lateral sclerosis. PLoS ONE 7:e36000. doi: 10.1371/journal.pone.0036000.
    • (2012) PLoS ONE , vol.7
    • Gerber, Y.N.1    Sabourin, J.C.2    Rabano, M.3    Vivanco, M.D.4    Perrin, F.E.5
  • 31
    • 36849005504 scopus 로고    scopus 로고
    • Exogenous delivery of heat shock protein 70 increases lifespan in a mouse model of amyotrophic lateral sclerosis
    • Gifondorwa, D. J., M. B. Robinson, C. D. Hayes, A. R. Taylor, D. M. Prevette, R. W. Oppenheim, et al. 2007. Exogenous delivery of heat shock protein 70 increases lifespan in a mouse model of amyotrophic lateral sclerosis. J. Neurosci. 27:13173-13180.
    • (2007) J. Neurosci. , vol.27 , pp. 13173-13180
    • Gifondorwa, D.J.1    Robinson, M.B.2    Hayes, C.D.3    Taylor, A.R.4    Prevette, D.M.5    Oppenheim, R.W.6
  • 32
    • 84866181145 scopus 로고    scopus 로고
    • Administration of recombinant heat shock protein 70 delays peripheral muscle denervation in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis
    • doi: 10.1155/2012/170426.
    • Gifondorwa, D. J., R. Jimenz-Moreno, C. D. Hayes, H. Rouhani, M. B. Robinson, J. L. Strupe, et al. 2012. Administration of recombinant heat shock protein 70 delays peripheral muscle denervation in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Neurol. Res. Int. 2012:170426. doi: 10.1155/2012/170426.
    • (2012) Neurol. Res. Int. , vol.2012 , pp. 170426
    • Gifondorwa, D.J.1    Jimenz-Moreno, R.2    Hayes, C.D.3    Rouhani, H.4    Robinson, M.B.5    Strupe, J.L.6
  • 33
    • 0026529968 scopus 로고
    • Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis
    • Gonatas, N. K., A. Stieber, Z. Mourelatos, Y. Chen, J. O. Gonatas, S. H. Appel, et al. 1992. Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis. Am. J. Pathol. 140:731-737.
    • (1992) Am. J. Pathol. , vol.140 , pp. 731-737
    • Gonatas, N.K.1    Stieber, A.2    Mourelatos, Z.3    Chen, Y.4    Gonatas, J.O.5    Appel, S.H.6
  • 34
    • 33744531110 scopus 로고    scopus 로고
    • Fragmentation of the Golgi apparatus in neurodegenerative diseases and cell death
    • Gonatas, N. K., A. Stieber, and J. O. Gonatas. 2006. Fragmentation of the Golgi apparatus in neurodegenerative diseases and cell death. J. Neurol. Sci. 246:21-30.
    • (2006) J. Neurol. Sci. , vol.246 , pp. 21-30
    • Gonatas, N.K.1    Stieber, A.2    Gonatas, J.O.3
  • 35
    • 36448939830 scopus 로고    scopus 로고
    • Synaptic dysfunction in disease and following injury in the developing and adult nervous system: caveats in the choice of therapeutic intervention
    • Gould, T. W., and R. W. Oppenheim. 2007. Synaptic dysfunction in disease and following injury in the developing and adult nervous system: caveats in the choice of therapeutic intervention. Neurosci. Biobehav. Rev. 31:1073-1087.
    • (2007) Neurosci. Biobehav. Rev. , vol.31 , pp. 1073-1087
    • Gould, T.W.1    Oppenheim, R.W.2
  • 36
    • 33748261830 scopus 로고    scopus 로고
    • Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS
    • Gould, T. W., R. R. Buss, S. Vinsant, D. Prevette, W. Sun, C. M. Knudson, et al. 2006. Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS. J. Neurosci. 26:8774-8786.
    • (2006) J. Neurosci. , vol.26 , pp. 8774-8786
    • Gould, T.W.1    Buss, R.R.2    Vinsant, S.3    Prevette, D.4    Sun, W.5    Knudson, C.M.6
  • 37
    • 0028284779 scopus 로고
    • Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation
    • Gurney, M. E., H. Pu, A. Y. Chiu, M. C. Dal Canto, C. Y. Polchow, D. D. Alexander, et al. 1994. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264:1772-1775.
    • (1994) Science , vol.264 , pp. 1772-1775
    • Gurney, M.E.1    Pu, H.2    Chiu, A.Y.3    Dal Canto, M.C.4    Polchow, C.Y.5    Alexander, D.D.6
  • 38
    • 0037734370 scopus 로고    scopus 로고
    • Mutations in dynein link motor neuron degeneration to defects in retrograde transport
    • Hafezparast, M., R. Klocke, C. Ruhrberg, A. Marquardt, A. Ahmad-Annuar, S. Bowen, et al. 2003. Mutations in dynein link motor neuron degeneration to defects in retrograde transport. Science 300:808-810.
    • (2003) Science , vol.300 , pp. 808-810
    • Hafezparast, M.1    Klocke, R.2    Ruhrberg, C.3    Marquardt, A.4    Ahmad-Annuar, A.5    Bowen, S.6
  • 39
    • 0032125587 scopus 로고    scopus 로고
    • Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS
    • Hall, E. D., J. A. Oostveen, and M. E. Gurney. 1998. Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS. Glia 23:249-256.
    • (1998) Glia , vol.23 , pp. 249-256
    • Hall, E.D.1    Oostveen, J.A.2    Gurney, M.E.3
  • 42
    • 0344240348 scopus 로고    scopus 로고
    • G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes
    • G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes. BMC Neurosci. 4:16.
    • (2003) BMC Neurosci. , vol.4 , pp. 16
    • Higgins, C.M.1    Jung, C.2    Xu, Z.3
  • 43
    • 36749067790 scopus 로고    scopus 로고
    • Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis
    • Ilieva, E. V., V. Ayala, M. Jové, E. Dalfó, D. Cacabelos, M. Povedano, et al. 2007. Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis. Brain 130:3111-3123.
    • (2007) Brain , vol.130 , pp. 3111-3123
    • Ilieva, E.V.1    Ayala, V.2    Jové, M.3    Dalfó, E.4    Cacabelos, D.5    Povedano, M.6
  • 44
    • 50449101429 scopus 로고    scopus 로고
    • Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death
    • Ilieva, H. S., K. Yamanaka, S. Malkmus, O. Kakinohana, T. Yaksh, M. Marsala, et al. 2008. Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc. Natl. Acad. Sci. USA 105:12599-12604.
    • (2008) Proc. Natl. Acad. Sci. USA , vol.105 , pp. 12599-12604
    • Ilieva, H.S.1    Yamanaka, K.2    Malkmus, S.3    Kakinohana, O.4    Yaksh, T.5    Marsala, M.6
  • 45
    • 74049164709 scopus 로고    scopus 로고
    • Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
    • Ilieva, H., M. Polymenidou, and D. W. Cleveland. 2009. Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J. Cell Biol. 187:761-772.
    • (2009) J. Cell Biol. , vol.187 , pp. 761-772
    • Ilieva, H.1    Polymenidou, M.2    Cleveland, D.W.3
  • 46
    • 0034520591 scopus 로고    scopus 로고
    • Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1
    • Jaarsma, D., E. D. Haasdijk, J. A. Grashorn, R. Hawkins, W. van Duijn, H. W. Verspaget, et al. 2000. Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1. Neurobiol. Dis. 7(6 Pt B):623-643.
    • (2000) Neurobiol. Dis. , vol.7 , Issue.6 PART B , pp. 623-643
    • Jaarsma, D.1    Haasdijk, E.D.2    Grashorn, J.A.3    Hawkins, R.4    van Duijn, W.5    Verspaget, H.W.6
  • 47
    • 0034821922 scopus 로고    scopus 로고
    • CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations
    • Jaarsma, D., F. Rognoni, W. van Duijn, H. W. Verspaget, E. D. Haasdijk, and J. C. Holstege. 2001. CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations. Acta Neuropathol. 102:293-305.
    • (2001) Acta Neuropathol. , vol.102 , pp. 293-305
    • Jaarsma, D.1    Rognoni, F.2    van Duijn, W.3    Verspaget, H.W.4    Haasdijk, E.D.5    Holstege, J.C.6
  • 48
    • 0842290736 scopus 로고    scopus 로고
    • Axonal defects in mouse models of motoneuron disease
    • Jablonka, S., S. Wiese, and M. Sendtner. 2004. Axonal defects in mouse models of motoneuron disease. J. Neurobiol. 58:272-286.
    • (2004) J. Neurobiol. , vol.58 , pp. 272-286
    • Jablonka, S.1    Wiese, S.2    Sendtner, M.3
  • 49
    • 77952312535 scopus 로고    scopus 로고
    • Increased superoxide in vivo accelerates age-associated muscle atrophy through mitochondrial dysfunction and neuromuscular junction degeneration
    • Jang, Y. C., M. S. Lustgarten, Y. Liu, F. L. Muller, A. Bhattacharya, H. Liang, et al. 2010. Increased superoxide in vivo accelerates age-associated muscle atrophy through mitochondrial dysfunction and neuromuscular junction degeneration. FASEB J. 24:1376-1390.
    • (2010) FASEB J. , vol.24 , pp. 1376-1390
    • Jang, Y.C.1    Lustgarten, M.S.2    Liu, Y.3    Muller, F.L.4    Bhattacharya, A.5    Liang, H.6
  • 50
    • 72849120555 scopus 로고    scopus 로고
    • Progressive changes in synaptic inputs to motoneurons in adult sacral spinal cord of a mouse model of amyotrophic lateral sclerosis
    • Jiang, M., J. E. Schuster, R. Fu, T. Siddique, and C. J. Heckman. 2009. Progressive changes in synaptic inputs to motoneurons in adult sacral spinal cord of a mouse model of amyotrophic lateral sclerosis. J. Neurosci. 29:15031-15038.
    • (2009) J. Neurosci. , vol.29 , pp. 15031-15038
    • Jiang, M.1    Schuster, J.E.2    Fu, R.3    Siddique, T.4    Heckman, C.J.5
  • 51
    • 0024573916 scopus 로고
    • Ultrastructure of axotomized alpha and gamma motoneurons in the cat thoracic spinal cord
    • Johnston, I. P., and T. A. Sears. 1989. Ultrastructure of axotomized alpha and gamma motoneurons in the cat thoracic spinal cord. Neuropathol. Appl. Neurobiol. 15:149-163.
    • (1989) Neuropathol. Appl. Neurobiol. , vol.15 , pp. 149-163
    • Johnston, I.P.1    Sears, T.A.2
  • 52
    • 34247505633 scopus 로고    scopus 로고
    • ALS: astrocytes move in as deadly neighbors
    • Jullien, J. P. 2007. ALS: astrocytes move in as deadly neighbors. Nat. Neurosci. 10:535-537.
    • (2007) Nat. Neurosci. , vol.10 , pp. 535-537
    • Jullien, J.P.1
  • 53
    • 66149156941 scopus 로고    scopus 로고
    • ER stress and unfolded protein response in amyotrophic lateral sclerosis
    • Kanekura, K., H. Suzuki, S. Aiso, and M. Matsuoka. 2009. ER stress and unfolded protein response in amyotrophic lateral sclerosis. Mol. Neurobiol. 39:81-89.
    • (2009) Mol. Neurobiol. , vol.39 , pp. 81-89
    • Kanekura, K.1    Suzuki, H.2    Aiso, S.3    Matsuoka, M.4
  • 54
    • 78449269355 scopus 로고    scopus 로고
    • NG2+ CNS glial progenitors remain committed to the oligodendrocyte lineage in postnatal life and following neurodegeneration
    • Kang, S. H., M. Fukaya, J. K. Yang, J. D. Rothstein, and D. E. Bergles. 2010. NG2+ CNS glial progenitors remain committed to the oligodendrocyte lineage in postnatal life and following neurodegeneration. Neuron 68:668-681.
    • (2010) Neuron , vol.68 , pp. 668-681
    • Kang, S.H.1    Fukaya, M.2    Yang, J.K.3    Rothstein, J.D.4    Bergles, D.E.5
  • 55
    • 21344472483 scopus 로고    scopus 로고
    • A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice
    • Kieran, D., M. Hafezparast, S. Bohnert, J. R. T. Dick, J. Martin, G. Schiavo, et al. 2005. A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J. Cell Biol. 169:561-567.
    • (2005) J. Cell Biol. , vol.169 , pp. 561-567
    • Kieran, D.1    Hafezparast, M.2    Bohnert, S.3    Dick, J.R.T.4    Martin, J.5    Schiavo, G.6
  • 57
    • 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 Z. Xu. 1998. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J. Neurosci. 18:3241-3250.
    • (1998) J. Neurosci. , vol.18 , pp. 3241-3250
    • Kong, J.1    Xu, Z.2
  • 58
    • 0030756459 scopus 로고    scopus 로고
    • Bcl-2: prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis
    • Kostic, V., V. Jackson-Lewis, F. de Bilbao, M. Dubois-Dauphin, and S. Przedborski. 1997. Bcl-2: prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis. Science 277:559-562.
    • (1997) Science , vol.277 , pp. 559-562
    • Kostic, V.1    Jackson-Lewis, V.2    de Bilbao, F.3    Dubois-Dauphin, M.4    Przedborski, S.5
  • 59
    • 79960563254 scopus 로고    scopus 로고
    • Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons
    • Lee, Y. I., M. Mikesh, I. Smith, M. Rimer, and W. Thompson. 2011. Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons. Dev. Biol. 356:432-444.
    • (2011) Dev. Biol. , vol.356 , pp. 432-444
    • Lee, Y.I.1    Mikesh, M.2    Smith, I.3    Rimer, M.4    Thompson, W.5
  • 60
    • 0028915940 scopus 로고
    • C-terminals on motoneurons: electron microscope localization of cholinergic markers in adult rats and antibody-induced depletion in neonates
    • Li, W., P. A. Ochalski, S. Brimijoin, L. M. Jordan, and J. I. Nagy. 1995. C-terminals on motoneurons: electron microscope localization of cholinergic markers in adult rats and antibody-induced depletion in neonates. Neuroscience 65:879-891.
    • (1995) Neuroscience , vol.65 , pp. 879-891
    • Li, W.1    Ochalski, P.A.2    Brimijoin, S.3    Jordan, L.M.4    Nagy, J.I.5
  • 62
    • 77955614345 scopus 로고    scopus 로고
    • Susceptibility to neurodegeneration in a glaucoma is modified by Bax gene dosage
    • Libby, R. T., Y. Li, O. V. Savinova, J. Barter, R. S. Smith, R. W. Nickells, et al. 2005. Susceptibility to neurodegeneration in a glaucoma is modified by Bax gene dosage. PLoS Genet. 1:17-26.
    • (2005) PLoS Genet. , vol.1 , pp. 17-26
    • Libby, R.T.1    Li, Y.2    Savinova, O.V.3    Barter, J.4    Smith, R.S.5    Nickells, R.W.6
  • 64
    • 77956997715 scopus 로고    scopus 로고
    • Mechanisms of synapse and dendrite maintenance and their disruption in psychiatric and neurodegenerative disorders
    • Lin, Y. C., and A. J. Koleske. 2010. Mechanisms of synapse and dendrite maintenance and their disruption in psychiatric and neurodegenerative disorders. Annu. Rev. Neurosci. 33:349-378.
    • (2010) Annu. Rev. Neurosci. , vol.33 , pp. 349-378
    • Lin, Y.C.1    Koleske, A.J.2
  • 65
    • 0026319184 scopus 로고
    • Inducible isoform of HSP70 is constitutively expressed in a muscle fiber type specific pattern
    • Locke, M., E. G. Noble, and B. G. Atkinson. 1991. Inducible isoform of HSP70 is constitutively expressed in a muscle fiber type specific pattern. Am. J. Physiol. 261:C774-C779.
    • (1991) Am. J. Physiol. , vol.261
    • Locke, M.1    Noble, E.G.2    Atkinson, B.G.3
  • 66
    • 0028008086 scopus 로고
    • Shifts in type I fiber proportion in rat hindlimb muscle are accompanied by changes in HSP72 content
    • Locke, M., B. G. Atkinson, R. M. Tanguay, and E. G. Noble. 1994. Shifts in type I fiber proportion in rat hindlimb muscle are accompanied by changes in HSP72 content. Am. J. Physiol. 266:C1240-C1246.
    • (1994) Am. J. Physiol. , vol.266
    • Locke, M.1    Atkinson, B.G.2    Tanguay, R.M.3    Noble, E.G.4
  • 67
    • 79151485570 scopus 로고    scopus 로고
    • Mitochondrial trafficking and the provision of energy and calcium buffering at excitatory synapses
    • MacAskill, A. F., T. A. Atkin, and J. T. Kittler. 2010. Mitochondrial trafficking and the provision of energy and calcium buffering at excitatory synapses. Eur. J. Neurosci. 32:231-240.
    • (2010) Eur. J. Neurosci. , vol.32 , pp. 231-240
    • MacAskill, A.F.1    Atkin, T.A.2    Kittler, J.T.3
  • 68
    • 66749104344 scopus 로고    scopus 로고
    • Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis
    • Magrané, J., and G. Manfredi. 2009. Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis. Antioxid. Redox Signal. 11:1615-1626.
    • (2009) Antioxid. Redox Signal. , vol.11 , pp. 1615-1626
    • Magrané, J.1    Manfredi, G.2
  • 69
    • 79960836827 scopus 로고    scopus 로고
    • Evolution of gait abnormalities in SOD1(G93A) transgenic mice
    • doi: 10.1016/j.brainres.2011.06.033.
    • Mancuso, R., S. Oliván, R. Osta, and X. Navarro. 2011. Evolution of gait abnormalities in SOD1(G93A) transgenic mice. Brain Res. 1406:65-73. doi: 10.1016/j.brainres.2011.06.033.
    • (2011) Brain Res. , vol.1406 , pp. 65-73
    • Mancuso, R.1    Oliván, S.2    Osta, R.3    Navarro, X.4
  • 70
    • 16844366080 scopus 로고    scopus 로고
    • Mitochondrial dysfunction and its role in motor neuron degeneration in ALS
    • Manfredi, G., and Z. Xu. 2005. Mitochondrial dysfunction and its role in motor neuron degeneration in ALS. Mitochondrion 5:77-87.
    • (2005) Mitochondrion , vol.5 , pp. 77-87
    • Manfredi, G.1    Xu, Z.2
  • 71
    • 84858259015 scopus 로고    scopus 로고
    • Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis
    • Marinkovic, P., M. S. Reuter, M. S. Brill, L. Godinho, M. Kerschensteiner, and T. Misgeld. 2012. Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. USA 109:4296-4301.
    • (2012) Proc. Natl. Acad. Sci. USA , vol.109 , pp. 4296-4301
    • Marinkovic, P.1    Reuter, M.S.2    Brill, M.S.3    Godinho, L.4    Kerschensteiner, M.5    Misgeld, T.6
  • 72
    • 77956216847 scopus 로고    scopus 로고
    • Mitochondrial and cell death mechanisms in neurodegenerative diseases
    • Martin, L. J. 2010. Mitochondrial and cell death mechanisms in neurodegenerative diseases. Pharmaceuticals (Basel) 3:839-915.
    • (2010) Pharmaceuticals (Basel) , vol.3 , pp. 839-915
    • Martin, L.J.1
  • 73
    • 84856717207 scopus 로고    scopus 로고
    • Inhibitory synaptic regulation of motoneurons: a new target of disease mechanisms in amyotrophic lateral sclerosis
    • Martin, L. J., and Q. Chang. 2012. Inhibitory synaptic regulation of motoneurons: a new target of disease mechanisms in amyotrophic lateral sclerosis. Mol. Neurobiol. 45:30-42.
    • (2012) Mol. Neurobiol. , vol.45 , pp. 30-42
    • Martin, L.J.1    Chang, Q.2
  • 74
    • 0035945348 scopus 로고    scopus 로고
    • Peri-Golgi vesicles contain retrograde but not anterograde proteins consistent with the cisternal progression model of intra-Golgi transport
    • Martinez-Menárguez, J. A., R. Prekeris, V. M. Oorschot, R. Scheller, J. W. Slot, H. J. Geuze, et al. 2001. Peri-Golgi vesicles contain retrograde but not anterograde proteins consistent with the cisternal progression model of intra-Golgi transport. J. Cell Biol. 155:1213-1224.
    • (2001) J. Cell Biol. , vol.155 , pp. 1213-1224
    • Martinez-Menárguez, J.A.1    Prekeris, R.2    Oorschot, V.M.3    Scheller, R.4    Slot, J.W.5    Geuze, H.J.6
  • 76
    • 80051830113 scopus 로고    scopus 로고
    • Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS)
    • doi: 10.1371/journal.pone.0023244.
    • Mead, R. J., E. J. Bennett, A. J. Kennerley, P. Sharp, C. Sunyach, P. Kasher, et al. 2011. Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS). PLoS ONE 6:e23244. doi: 10.1371/journal.pone.0023244.
    • (2011) PLoS ONE , vol.6
    • Mead, R.J.1    Bennett, E.J.2    Kennerley, A.J.3    Sharp, P.4    Sunyach, C.5    Kasher, P.6
  • 77
    • 0037370914 scopus 로고    scopus 로고
    • Axonal damage: a key predictor of outcome in human CNS diseases
    • Medana, I. M., and M. M. Esiri. 2003. Axonal damage: a key predictor of outcome in human CNS diseases. Brain 126:515-530.
    • (2003) Brain , vol.126 , pp. 515-530
    • Medana, I.M.1    Esiri, M.M.2
  • 78
    • 79551663958 scopus 로고    scopus 로고
    • Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy
    • Mentis, G. Z., D. Blivis, W. Liu, E. Drobac, M. E. Crowder, L. Kong, et al. 2011. Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy. Neuron 69:453-467.
    • (2011) Neuron , vol.69 , pp. 453-467
    • Mentis, G.Z.1    Blivis, D.2    Liu, W.3    Drobac, E.4    Crowder, M.E.5    Kong, L.6
  • 79
    • 33746801906 scopus 로고    scopus 로고
    • ALS: life and death in a bad neighborhood
    • Monk, P. N., and P. J. Shaw. 2006. ALS: life and death in a bad neighborhood. Nat. Med. 12:885-887.
    • (2006) Nat. Med. , vol.12 , pp. 885-887
    • Monk, P.N.1    Shaw, P.J.2
  • 81
    • 0029890685 scopus 로고    scopus 로고
    • The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu, Zn superoxide dismutase becomes fragmented in early, preclinical stages
    • Mourelatos, Z., N. K. Gonatas, A. Stieber, M. E. Gurney, and M. C. Dal Canto. 1996. The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu, Zn superoxide dismutase becomes fragmented in early, preclinical stages. Proc. Natl. Acad. Sci. USA 93:5472-5477.
    • (1996) Proc. Natl. Acad. Sci. USA , vol.93 , pp. 5472-5477
    • Mourelatos, Z.1    Gonatas, N.K.2    Stieber, A.3    Gurney, M.E.4    Dal Canto, M.C.5
  • 82
    • 34247880172 scopus 로고    scopus 로고
    • Early decrease of mitochondrial DNA repair enzymes in spinal motor neurons of presymptomatic transgenic mice carrying a mutant SOD1 gene
    • Murakami, T., M. Nagai, K. Miyazaki, N. Morimoto, Y. Ohta, T. Kurata, et al. 2007. Early decrease of mitochondrial DNA repair enzymes in spinal motor neurons of presymptomatic transgenic mice carrying a mutant SOD1 gene. Brain Res. 1150:182-189.
    • (2007) Brain Res. , vol.1150 , pp. 182-189
    • Murakami, T.1    Nagai, M.2    Miyazaki, K.3    Morimoto, N.4    Ohta, Y.5    Kurata, T.6
  • 83
    • 34247475338 scopus 로고    scopus 로고
    • Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
    • Nagai, M., D. B. Re, T. Nagata, A. Chalazonitis, T. M. Jessell, H. Wichterle, et al. 2007. Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat. Neurosci. 10:615-622.
    • (2007) Nat. Neurosci. , vol.10 , pp. 615-622
    • Nagai, M.1    Re, D.B.2    Nagata, T.3    Chalazonitis, A.4    Jessell, T.M.5    Wichterle, H.6
  • 84
    • 37149031347 scopus 로고    scopus 로고
    • Increased ER stress during motor neuron degeneration in a transgenic mouse model of amyotrophic lateral sclerosis
    • Nagata, T., H. Ilieva, T. Murakami, M. Shiote, H. Narai, Y. Ohta, et al. 2007. Increased ER stress during motor neuron degeneration in a transgenic mouse model of amyotrophic lateral sclerosis. Neurol. Res. 29:767-771.
    • (2007) Neurol. Res. , vol.29 , pp. 767-771
    • Nagata, T.1    Ilieva, H.2    Murakami, T.3    Shiote, M.4    Narai, H.5    Ohta, Y.6
  • 85
    • 0027329088 scopus 로고
    • Evidence for the cholinergic nature of C-terminals associated with subsurface cisterns in alpha-motoneurons of rat
    • Nagy, J. I., T. Yamamoto, and L. M. Jordan. 1993. Evidence for the cholinergic nature of C-terminals associated with subsurface cisterns in alpha-motoneurons of rat. Synapse 15:17-32.
    • (1993) Synapse , vol.15 , pp. 17-32
    • Nagy, J.I.1    Yamamoto, T.2    Jordan, L.M.3
  • 86
    • 44849124411 scopus 로고    scopus 로고
    • ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
    • Nishitoh, H., H. Kadowaki, A. Nagai, T. Maruyama, T. Yokota, H. Fukutomi, et al. 2008. ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. Genes Dev. 22:1451-1464.
    • (2008) Genes Dev. , vol.22 , pp. 1451-1464
    • Nishitoh, H.1    Kadowaki, H.2    Nagai, A.3    Maruyama, T.4    Yokota, T.5    Fukutomi, H.6
  • 87
    • 84893485909 scopus 로고    scopus 로고
    • Programmed cell death and neurotrophic factors
    • L. Squire, D. Berg, F. E. Bloom, S. duLac, A. Ghosh, and N. C. Spitzer, eds. 4th ed. Academic Press, San Diego, CA.
    • Oppenheim, R. W., C. Milligan, and C. von Bartheld. 2013. Programmed cell death and neurotrophic factors. Pp. 405-435 in L. Squire, D. Berg, F. E. Bloom, S. duLac, A. Ghosh, and N. C. Spitzer, eds. Fundamental neuroscience. 4th ed. Academic Press, San Diego, CA.
    • (2013) Fundamental neuroscience , pp. 405-435
    • Oppenheim, R.W.1    Milligan, C.2    von Bartheld, C.3
  • 89
    • 33750339228 scopus 로고    scopus 로고
    • A network dysfunction perspective on neurodegenerative diseases
    • Palop, J. J., J. Chin, and L. Mucke. 2006. A network dysfunction perspective on neurodegenerative diseases. Nature 443:768-773.
    • (2006) Nature , vol.443 , pp. 768-773
    • Palop, J.J.1    Chin, J.2    Mucke, L.3
  • 91
    • 1942423576 scopus 로고    scopus 로고
    • Astrocytic production of nerve growth factor in motor neuron apoptosis: implications for amyotrophic lateral sclerosis
    • Pehar, M., P. Cassina, M. R. Vargas, R. Castellanos, L. Viera, J. S. Beckman, et al. 2004. Astrocytic production of nerve growth factor in motor neuron apoptosis: implications for amyotrophic lateral sclerosis. J. Neurochem. 89:464-473.
    • (2004) J. Neurochem. , vol.89 , pp. 464-473
    • Pehar, M.1    Cassina, P.2    Vargas, M.R.3    Castellanos, R.4    Viera, L.5    Beckman, J.S.6
  • 93
    • 68549121208 scopus 로고    scopus 로고
    • A switch in retrograde signaling from survival to stress in rapid-onset neurodegeneration
    • Perlson, E., G. B. Jeong, J. L. Ross, R. Dixit, K. E. Wallace, R. G. Kalb, et al. 2009. A switch in retrograde signaling from survival to stress in rapid-onset neurodegeneration. J. Neurosci. 29:9903-9917.
    • (2009) J. Neurosci. , vol.29 , pp. 9903-9917
    • Perlson, E.1    Jeong, G.B.2    Ross, J.L.3    Dixit, R.4    Wallace, K.E.5    Kalb, R.G.6
  • 94
    • 59149096854 scopus 로고    scopus 로고
    • Increase in presynaptic territory of C-terminals on lumbar motoneurons of G93A SOD1 mice during disease progression
    • Pullen, A. H., and D. Athanasiou. 2009. Increase in presynaptic territory of C-terminals on lumbar motoneurons of G93A SOD1 mice during disease progression. Eur. J. Neurosci. 29:551-561.
    • (2009) Eur. J. Neurosci. , vol.29 , pp. 551-561
    • Pullen, A.H.1    Athanasiou, D.2
  • 95
    • 33344462702 scopus 로고    scopus 로고
    • Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF
    • Pun, S., A. F. Santos, S. Saxena, L. Xu, and P. Caroni. 2006. Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF. Nat. Neurosci. 9:408-419.
    • (2006) Nat. Neurosci. , vol.9 , pp. 408-419
    • Pun, S.1    Santos, A.F.2    Saxena, S.3    Xu, L.4    Caroni, P.5
  • 96
    • 0037012844 scopus 로고    scopus 로고
    • Axonal self-destruction and neurodegeneration
    • Raff, M. C., A. V. Whitmore, and J. T. Finn. 2002. Axonal self-destruction and neurodegeneration. Science 296:868-871.
    • (2002) Science , vol.296 , pp. 868-871
    • Raff, M.C.1    Whitmore, A.V.2    Finn, J.T.3
  • 97
    • 0038632271 scopus 로고    scopus 로고
    • Defective neurofilament transport in mouse models of amyotrophic lateral sclerosis: a review
    • Rao, M. V., and R. A. Nixon. 2003. Defective neurofilament transport in mouse models of amyotrophic lateral sclerosis: a review. Neurochem. Res. 28:1041-1047.
    • (2003) Neurochem. Res. , vol.28 , pp. 1041-1047
    • Rao, M.V.1    Nixon, R.A.2
  • 98
    • 71849085539 scopus 로고    scopus 로고
    • Mitochondrial trafficking and morphology in neuronal injury
    • Rintoul, G. L., and I. J. Reynolds. 2009. Mitochondrial trafficking and morphology in neuronal injury. Biochim. Biophys. Acta 1802:143-150.
    • (2009) Biochim. Biophys. Acta , vol.1802 , pp. 143-150
    • Rintoul, G.L.1    Reynolds, I.J.2
  • 99
    • 84893449560 scopus 로고    scopus 로고
    • Mechanisms of the motoneuron stress response and its relevance in neurodegeneration
    • A. S. McNeill, ed. Nova Science Publishers, Inc., Hauppauge, NY.
    • Robinson, M. B., D. J. Gifondorwa, and C. Milligan. 2011. Mechanisms of the motoneuron stress response and its relevance in neurodegeneration. Pp. 45-62 in A. S. McNeill, ed. Neurodegeneration: theory, disorders and treatments. Nova Science Publishers, Inc., Hauppauge, NY.
    • (2011) Neurodegeneration: theory, disorders and treatments , pp. 45-62
    • Robinson, M.B.1    Gifondorwa, D.J.2    Milligan, C.3
  • 101
    • 0029441620 scopus 로고
    • Excitotoxic mechanisms in the pathogenesis of amyotrophic lateral sclerosis
    • discussion 21-27.
    • Rothstein, J. D. 1995. Excitotoxic mechanisms in the pathogenesis of amyotrophic lateral sclerosis. Adv. Neurol. 68:7-20; discussion 21-27.
    • (1995) Adv. Neurol. , vol.68 , pp. 7-20
    • Rothstein, J.D.1
  • 103
    • 0026061367 scopus 로고
    • The plasticity of skeletal muscle: effects of neuromuscular activity
    • Roy, R. R., K. M. Baldwin, and V. R. Edgerton. 1991. The plasticity of skeletal muscle: effects of neuromuscular activity. Exerc. Sport Sci. Rev. 19:269-312.
    • (1991) Exerc. Sport Sci. Rev. , vol.19 , pp. 269-312
    • Roy, R.R.1    Baldwin, K.M.2    Edgerton, V.R.3
  • 104
    • 0028865136 scopus 로고
    • Bcl-2 overexpression prevents motoneuron cell body loss but not axonal degeneration in a mouse model of neurodegenerative disease
    • Sagot, Y., M. Dubois-Dauphin, S. A. Tan, F. de Bibao, P. Aebischer, J.-C. Martinou, et al. 1995. Bcl-2 overexpression prevents motoneuron cell body loss but not axonal degeneration in a mouse model of neurodegenerative disease. J. Neurosci. 15:7727-7733.
    • (1995) J. Neurosci. , vol.15 , pp. 7727-7733
    • Sagot, Y.1    Dubois-Dauphin, M.2    Tan, S.A.3    de Bibao, F.4    Aebischer, P.5    Martinou, J.-C.6
  • 105
    • 25444522220 scopus 로고    scopus 로고
    • Microglia as potential contributors to motor neuron injury in amyotrophic lateral sclerosis
    • Sargsyan, S. A., P. N. Monk, and P. J. Shaw. 2005. Microglia as potential contributors to motor neuron injury in amyotrophic lateral sclerosis. Glia 51:241-253.
    • (2005) Glia , vol.51 , pp. 241-253
    • Sargsyan, S.A.1    Monk, P.N.2    Shaw, P.J.3
  • 106
    • 2342418195 scopus 로고    scopus 로고
    • Slow component of axonal transport is impaired in the proximal axon of transgenic mice with a G93A mutant SOD1 gene
    • Sasaki, S., H. Warita, K. Abe, and M. Iwata. 2004. Slow component of axonal transport is impaired in the proximal axon of transgenic mice with a G93A mutant SOD1 gene. Acta Neuropathol. 107:452-460.
    • (2004) Acta Neuropathol. , vol.107 , pp. 452-460
    • Sasaki, S.1    Warita, H.2    Abe, K.3    Iwata, M.4
  • 107
  • 108
    • 34748819496 scopus 로고    scopus 로고
    • Mechanisms of axon degeneration: from development to disease
    • Saxena, S., and P. Caroni. 2007. Mechanisms of axon degeneration: from development to disease. Prog. Neurobiol. 83:174-191.
    • (2007) Prog. Neurobiol. , vol.83 , pp. 174-191
    • Saxena, S.1    Caroni, P.2
  • 109
    • 67349164383 scopus 로고    scopus 로고
    • A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice
    • Saxena, S., E. Cabuy, and P. Caroni. 2009. A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat. Neurosci. 12:627-636.
    • (2009) Nat. Neurosci. , vol.12 , pp. 627-636
    • Saxena, S.1    Cabuy, E.2    Caroni, P.3
  • 110
    • 23744445580 scopus 로고    scopus 로고
    • A compensatory subpopulation of motor neurons in a mouse model of amyotrophic lateral sclerosis
    • Schaefer, A. M., J. R. Sanes, and J. W. Lichtman. 2005. A compensatory subpopulation of motor neurons in a mouse model of amyotrophic lateral sclerosis. J. Comp. Neurol. 490:209-219.
    • (2005) J. Comp. Neurol. , vol.490 , pp. 209-219
    • Schaefer, A.M.1    Sanes, J.R.2    Lichtman, J.W.3
  • 111
    • 34548081175 scopus 로고    scopus 로고
    • Progressive motor neuronopathy: a critical role of the tubulin chaperone TBCE in axonal tubulin routing from the Golgi apparatus
    • Schaefer, M. K., H. Schmalbruch, E. Buhler, C. Lopez, N. Martin, J. L. Guénet, and G. Haase. 2007. Progressive motor neuronopathy: a critical role of the tubulin chaperone TBCE in axonal tubulin routing from the Golgi apparatus. J. Neurosci. 27:8779-8789.
    • (2007) J. Neurosci. , vol.27 , pp. 8779-8789
    • Schaefer, M.K.1    Schmalbruch, H.2    Buhler, E.3    Lopez, C.4    Martin, N.5    Guénet, J.L.6    Haase, G.7
  • 112
    • 0029812020 scopus 로고    scopus 로고
    • Reactive astrogliosis of the spinal cord in amyotrophic lateral sclerosis
    • Schiffer, D., S. Cordera, P. Cavalla, and A. Migheli. 1996. Reactive astrogliosis of the spinal cord in amyotrophic lateral sclerosis. J. Neurol. Sci. 139(Suppl.):27-33.
    • (1996) J. Neurol. Sci. , vol.139 , Issue.SUPPL. , pp. 27-33
    • Schiffer, D.1    Cordera, S.2    Cavalla, P.3    Migheli, A.4
  • 113
    • 58149376487 scopus 로고    scopus 로고
    • Synaptic ultrastructural alterations anticipate the development of neuroaxonal dystrophy in sympathetic ganglia of aged and diabetic mice
    • Schmidt, R. E., C. A. Parvin, and K. G. Green. 2008. Synaptic ultrastructural alterations anticipate the development of neuroaxonal dystrophy in sympathetic ganglia of aged and diabetic mice. J. Neuropathol. Exp. Neurol. 67:1166-1186.
    • (2008) J. Neuropathol. Exp. Neurol. , vol.67 , pp. 1166-1186
    • Schmidt, R.E.1    Parvin, C.A.2    Green, K.G.3
  • 114
    • 79959305691 scopus 로고    scopus 로고
    • Mitochondria: the next (neurode)generation
    • Schon, E. A., and S. Przedborski. 2011. Mitochondria: the next (neurode)generation. Neuron 70:1033-1053.
    • (2011) Neuron , vol.70 , pp. 1033-1053
    • Schon, E.A.1    Przedborski, S.2
  • 115
    • 77956224840 scopus 로고    scopus 로고
    • Mitochondrial dysfunction is a converging point of multiple pathological pathways in amyotrophic lateral sclerosis
    • Shi, P., Y. Wei, J. Zhang, J. Gal, and H. Zhu. 2010. Mitochondrial dysfunction is a converging point of multiple pathological pathways in amyotrophic lateral sclerosis. J. Alzheimers Dis. 20(Suppl. 2):S311-S324.
    • (2010) J. Alzheimers Dis. , vol.20 , Issue.SUPPL. 2
    • Shi, P.1    Wei, Y.2    Zhang, J.3    Gal, J.4    Zhu, H.5
  • 116
    • 0030026832 scopus 로고    scopus 로고
    • Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis
    • Siklós, L., J. Engelhardt, Y. Harati, R. G. Smith, F. Joó, and S. H. Appel. 1996. Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis. Ann. Neurol. 39:203-216.
    • (1996) Ann. Neurol. , vol.39 , pp. 203-216
    • Siklós, L.1    Engelhardt, J.2    Harati, Y.3    Smith, R.G.4    Joó, F.5    Appel, S.H.6
  • 117
    • 0031887308 scopus 로고    scopus 로고
    • The fragmented neuronal Golgi apparatus in amyotrophic lateral sclerosis includes the trans-Golgi-network: functional implications
    • Stieber, A., Y. Chen, S. Wei, Z. Mourelatos, J. Gonatas, K. Okamoto, and N. K. Gonatas. 1998. The fragmented neuronal Golgi apparatus in amyotrophic lateral sclerosis includes the trans-Golgi-network: functional implications. Acta Neuropathol. 95:245-253.
    • (1998) Acta Neuropathol. , vol.95 , pp. 245-253
    • Stieber, A.1    Chen, Y.2    Wei, S.3    Mourelatos, Z.4    Gonatas, J.5    Okamoto, K.6    Gonatas, N.K.7
  • 118
    • 37549065020 scopus 로고    scopus 로고
    • GDNF secreting human neural progenitor cells protect dying motor neurons, but not their projection to muscle, in a rat model of familial ALS
    • Suzuki, M., J. McHaugh, C. Tork, B. Shelley, S. M. Klein, P. Aebischer, et al. 2007. GDNF secreting human neural progenitor cells protect dying motor neurons, but not their projection to muscle, in a rat model of familial ALS. PLoS ONE 8:1-14.
    • (2007) PLoS ONE , vol.8 , pp. 1-14
    • Suzuki, M.1    McHaugh, J.2    Tork, C.3    Shelley, B.4    Klein, S.M.5    Aebischer, P.6
  • 119
    • 19444363520 scopus 로고    scopus 로고
    • Protein retention in the endoplasmic reticulum, blockade of programmed cell death and autophagy selectively occur in spinal cord motoneurons after glutamate receptor-mediated injury
    • Tarabal, O., J. Calderó, C. Casas, R. W. Oppenheim, and J. E. Esquerda. 2005. Protein retention in the endoplasmic reticulum, blockade of programmed cell death and autophagy selectively occur in spinal cord motoneurons after glutamate receptor-mediated injury. Mol. Cell. Neurosci. 29:283-298.
    • (2005) Mol. Cell. Neurosci. , vol.29 , pp. 283-298
    • Tarabal, O.1    Calderó, J.2    Casas, C.3    Oppenheim, R.W.4    Esquerda, J.E.5
  • 121
    • 0021338165 scopus 로고
    • The motor end-plate fine structure and ultrastructural localization of acetylcholine receptors in amyotrophic lateral sclerosis
    • Tsujihata, M., R. Hazama, T. Yoshimura, A. Satoh, M. Mori, and S. Nagataki. 1984. The motor end-plate fine structure and ultrastructural localization of acetylcholine receptors in amyotrophic lateral sclerosis. Muscle Nerve 7:243-249.
    • (1984) Muscle Nerve , vol.7 , pp. 243-249
    • Tsujihata, M.1    Hazama, R.2    Yoshimura, T.3    Satoh, A.4    Mori, M.5    Nagataki, S.6
  • 122
    • 77957090460 scopus 로고    scopus 로고
    • Attenuation of age-related changes in mouse neuromuscular synapses by caloric restriction and exercise
    • Valdez, G., J. C. Tapia, H. Kang, G. D. Clemenson Jr., F. H. Gage, J. W. Lichtman, et al. 2010. Attenuation of age-related changes in mouse neuromuscular synapses by caloric restriction and exercise. Proc. Natl. Acad. Sci. USA 107:14863-14868.
    • (2010) Proc. Natl. Acad. Sci. USA , vol.107 , pp. 14863-14868
    • Valdez, G.1    Tapia, J.C.2    Kang, H.3    Clemenson Jr, G.D.4    Gage, F.H.5    Lichtman, J.W.6
  • 123
    • 0014138019 scopus 로고
    • Electron microscopy of asphyxiated spinal cords of cats
    • Van Harreveld, A., and F. I. Khattab. 1967. Electron microscopy of asphyxiated spinal cords of cats. J. Neuropathol. Exp. Neurol. 26:521-536.
    • (1967) J. Neuropathol. Exp. Neurol. , vol.26 , pp. 521-536
    • Van Harreveld, A.1    Khattab, F.I.2
  • 124
    • 0037686243 scopus 로고    scopus 로고
    • Megamitochondria formation - physiology and pathology
    • Wakabayashi, T. 2002. Megamitochondria formation - physiology and pathology. J. Cell Mol. Med. 6:497-538.
    • (2002) J. Cell Mol. Med. , vol.6 , pp. 497-538
    • Wakabayashi, T.1
  • 125
    • 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 D. W. Cleveland. 1999. Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nat. Neurosci. 2:50-56.
    • (1999) Nat. Neurosci. , vol.2 , pp. 50-56
    • Williamson, T.L.1    Cleveland, D.W.2
  • 127
    • 0015426551 scopus 로고
    • Ultrastructural studies of the effects of various agents on the motor neurons of the spinal cord
    • Yates, R. D., and J. C. Yates. 1972. Ultrastructural studies of the effects of various agents on the motor neurons of the spinal cord. Am. J. Anat. 135:345-357.
    • (1972) Am. J. Anat. , vol.135 , pp. 345-357
    • Yates, R.D.1    Yates, J.C.2
  • 128
    • 84893445834 scopus 로고    scopus 로고
    • Identification of changes in muscle, neuromuscular junctions and spinal cord at early pre-symptomatic stages in the mutant SOD1 mouse model of ALS may provide novel insight for diagnosis and treatment development
    • Yoshikawa, M., S. Vinsant, C. M. Mansfield, R. J. Moreno, D. J. Gifondorwa, L. Pace, et al. 2009. Identification of changes in muscle, neuromuscular junctions and spinal cord at early pre-symptomatic stages in the mutant SOD1 mouse model of ALS may provide novel insight for diagnosis and treatment development. Soc. Neurosci. Abstr. 39:5.
    • (2009) Soc. Neurosci. Abstr. , vol.39 , pp. 5
    • Yoshikawa, M.1    Vinsant, S.2    Mansfield, C.M.3    Moreno, R.J.4    Gifondorwa, D.J.5    Pace, L.6
  • 129
    • 71149108487 scopus 로고    scopus 로고
    • A cluster of cholinergic premotor interneurons modulates mouse locomotor activity
    • Zagoraiou, L., T. Akay, J. F. Martin, R. M. Brownstone, T. M. Jessell, and G. B. Miles. 2009. A cluster of cholinergic premotor interneurons modulates mouse locomotor activity. Neuron 64:645-662.
    • (2009) Neuron , vol.64 , pp. 645-662
    • Zagoraiou, L.1    Akay, T.2    Martin, J.F.3    Brownstone, R.M.4    Jessell, T.M.5    Miles, G.B.6
  • 130
    • 41149097086 scopus 로고    scopus 로고
    • ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration
    • Zhong, Z., R. Deane, Z. Ali, M. Parisi, Y. Shapovalov, M. K. O'Banion, et al. 2008. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat. Neurosci. 11:420-422.
    • (2008) Nat. Neurosci. , vol.11 , pp. 420-422
    • Zhong, Z.1    Deane, R.2    Ali, Z.3    Parisi, M.4    Shapovalov, Y.5    O'Banion, M.K.6
  • 131
    • 70449433875 scopus 로고    scopus 로고
    • Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells
    • Zhong, Z., H. Ilieva, L. Hallagan, R. Bell, I. Singh, N. Paquette, et al. 2009. Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. J. Clin. Invest. 119:3437-3449.
    • (2009) J. Clin. Invest. , vol.119 , pp. 3437-3449
    • Zhong, Z.1    Ilieva, H.2    Hallagan, L.3    Bell, R.4    Singh, I.5    Paquette, N.6
  • 132
    • 58149173426 scopus 로고    scopus 로고
    • Neonatal neuronal circuitry shows hyperexcitable disturbance in a mouse model of the adult-onset neurodegenerative disease amyotrophic lateral sclerosis
    • van Zundert, B., M. H. Peuscher, M. Hynynen, A. Chen, R. L. Neve, R. H. Brown Jr., et al. 2008. Neonatal neuronal circuitry shows hyperexcitable disturbance in a mouse model of the adult-onset neurodegenerative disease amyotrophic lateral sclerosis. J. Neurosci. 28:10864-10874.
    • (2008) J. Neurosci. , vol.28 , pp. 10864-10874
    • van Zundert, B.1    Peuscher, M.H.2    Hynynen, M.3    Chen, A.4    Neve, R.L.5    Brown Jr, R.H.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.