메뉴 건너뛰기




Volumn 2015, Issue , 2015, Pages 5.67.1-5.67.21

Rodent models of amyotrophic lateral sclerosis

Author keywords

Aberrant protein homeostasis; Amyotrophic Lateral Sclerosis; Glia; Motor neuron; RNA processing alterations; Rodent models

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; TAR DNA BINDING PROTEIN; DNA BINDING PROTEIN; NERVE PROTEIN; SOD1 PROTEIN, HUMAN; SOD1 PROTEIN, MOUSE; SOD1 PROTEIN, RAT; SUPEROXIDE DISMUTASE; TDP-43 PROTEIN, HUMAN;

EID: 84949228570     PISSN: 19348282     EISSN: 19348290     Source Type: Journal    
DOI: 10.1002/0471141755.ph0567s69     Document Type: Article
Times cited : (183)

References (112)
  • 1
    • 84909953328 scopus 로고    scopus 로고
    • Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies
    • Alfieri, J.A., Pino, N.S., and Igaz, L.M. 2014. Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies. J. Neurosci. 34:15244-15259.
    • (2014) J. Neurosci , vol.34 , pp. 15244-15259
    • Alfieri, J.A.1    Pino, N.S.2    Igaz, L.M.3
  • 6
    • 0028097839 scopus 로고
    • A controlled trial of riluzole in amyotrophic lateral sclerosis
    • Bensimon, G., Lacomblez, L., and Meininger, V. 1994. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N. Engl. J. Med. 330:585-591.
    • (1994) N. Engl. J. Med , vol.330 , pp. 585-591
    • Bensimon, G.1    Lacomblez, L.2    Meininger, V.3
  • 7
    • 33749056809 scopus 로고    scopus 로고
    • ALS: A disease of motor neurons and their nonneuronal neighbors
    • Boillee, S., Vande Velde, C., and Cleveland, D.W. 2006a. ALS: A disease of motor neurons and their nonneuronal neighbors. Neuron 52:39-59.
    • (2006) Neuron , vol.52 , pp. 39-59
    • Boillee, S.1    Vande Velde, C.2    Cleveland, D.W.3
  • 9
    • 0030220998 scopus 로고    scopus 로고
    • Loss of functional prion protein: A role in prion disorders?
    • Borchelt, D.R. and Sisodia, S.S. 1996. Loss of functional prion protein: A role in prion disorders? Chem. Biol. 3:619-621.
    • (1996) Chem. Biol , vol.3 , pp. 619-621
    • Borchelt, D.R.1    Sisodia, S.S.2
  • 11
    • 83455230728 scopus 로고    scopus 로고
    • Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43
    • Caccamo, A., Majumder, S., and Oddo, S. 2012. Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43. Am. J. Pathol. 180:293-302.
    • (2012) Am. J. Pathol , vol.180 , pp. 293-302
    • Caccamo, A.1    Majumder, S.2    Oddo, S.3
  • 14
    • 77958012134 scopus 로고    scopus 로고
    • Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism
    • Chiang, P.M., Ling, J., Jeong, Y.H., Price, D.L., Aja, S.M., and Wong, P.C. 2010. Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism. Proc. Natl. Acad. Sci. U.S.A. 107:16320-16324.
    • (2010) Proc. Natl. Acad. Sci. U.S.A , vol.107 , pp. 16320-16324
    • Chiang, P.M.1    Ling, J.2    Jeong, Y.H.3    Price, D.L.4    Aja, S.M.5    Wong, P.C.6
  • 15
    • 0029991827 scopus 로고    scopus 로고
    • Colocalization of NOS and SOD1 in neurofilament accumulation within motor neurons of amyotrophic lateral sclerosis: an immunohistochemical study
    • Chou, S.M., Wang, H.S., and Komai, K. 1996. Colocalization of NOS and SOD1 in neurofilament accumulation within motor neurons of amyotrophic lateral sclerosis: an immunohistochemical study. J. Chem. Neuroanat. 10:249-258.
    • (1996) J. Chem. Neuroanat , vol.10 , pp. 249-258
    • Chou, S.M.1    Wang, H.S.2    Komai, K.3
  • 18
    • 0027946294 scopus 로고
    • Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis
    • Dal Canto, M.C. and Gurney, M.E. 1994. Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis. Am. J. Pathol. 145:1271-1279.
    • (1994) Am. J. Pathol , vol.145 , pp. 1271-1279
    • Dal Canto, M.C.1    Gurney, M.E.2
  • 27
    • 0034651102 scopus 로고    scopus 로고
    • Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration
    • Gong, Y.H., Parsadanian, A.S., Andreeva, A., Snider, W.D., and Elliott, J.L. 2000. Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration. J. Neurosci. 20:660-665.
    • (2000) J. Neurosci , vol.20 , pp. 660-665
    • Gong, Y.H.1    Parsadanian, A.S.2    Andreeva, A.3    Snider, W.D.4    Elliott, J.L.5
  • 34
    • 84855472423 scopus 로고    scopus 로고
    • Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats
    • Huang, C., Tong, J., Bi, F., Zhou, H., and Xia, X.G. 2012. Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats. J. Clin. Invest. 122:107-118.
    • (2012) J. Clin. Invest , vol.122 , pp. 107-118
    • Huang, C.1    Tong, J.2    Bi, F.3    Zhou, H.4    Xia, X.G.5
  • 37
    • 39849103473 scopus 로고    scopus 로고
    • Neuronspecific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice
    • Jaarsma, D., Teuling, E., Haasdijk, E.D., De Zeeuw, C.I., and Hoogenraad, C.C. 2008. Neuronspecific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice. J. Neurosci. 28:2075-2088.
    • (2008) J. Neurosci , vol.28 , pp. 2075-2088
    • Jaarsma, D.1    Teuling, E.2    Haasdijk, E.D.3    De Zeeuw, C.I.4    Hoogenraad, C.C.5
  • 41
    • 80051551180 scopus 로고    scopus 로고
    • SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments
    • Joyce, P.I., Fratta, P., Fisher, E.M., and AcevedoArozena, A. 2011. SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments. Mamm. Genome 22:420-448.
    • (2011) Mamm. Genome , vol.22 , pp. 420-448
    • Joyce, P.I.1    Fratta, P.2    Fisher, E.M.3    AcevedoArozena, A.4
  • 42
    • 0038147334 scopus 로고    scopus 로고
    • A prospective, randomized, placebo-controlled evaluation of corticoneuronal response to intrathecal BDNF therapy in ALS using magnetic resonance spectroscopy: Feasibility and results
    • Kalra, S., Genge, A., and Arnold, D.L. 2003. A prospective, randomized, placebo-controlled evaluation of corticoneuronal response to intrathecal BDNF therapy in ALS using magnetic resonance spectroscopy: Feasibility and results. Amyotroph. Lateral Scler. Other Motor Neuron Disord. 4:22-26.
    • (2003) Amyotroph. Lateral Scler. Other Motor Neuron Disord , vol.4 , pp. 22-26
    • Kalra, S.1    Genge, A.2    Arnold, D.L.3
  • 44
    • 16944366242 scopus 로고    scopus 로고
    • Reduction of CuZn-superoxide dismutase activity exacerbates neuronal cell injury and edema formation after transient focal cerebral ischemia
    • Kondo, T., Reaume, A.G., Huang, T.T., Carlson, E., Murakami, K., Chen, S.F., Hoffman, E.K., Scott, R.W., Epstein, C.J., and Chan, P.H. 1997. Reduction of CuZn-superoxide dismutase activity exacerbates neuronal cell injury and edema formation after transient focal cerebral ischemia. J. Neurosci. 17:4180-4189.
    • (1997) J. Neurosci , vol.17 , pp. 4180-4189
    • Kondo, T.1    Reaume, A.G.2    Huang, T.T.3    Carlson, E.4    Murakami, K.5    Chen, S.F.6    Hoffman, E.K.7    Scott, R.W.8    Epstein, C.J.9    Chan, P.H.10
  • 46
    • 0036406903 scopus 로고    scopus 로고
    • Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis
    • Kriz, J., Nguyen, M.D., and Julien, J.P. 2002. Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol. Dis. 10:268-278.
    • (2002) Neurobiol. Dis , vol.10 , pp. 268-278
    • Kriz, J.1    Nguyen, M.D.2    Julien, J.P.3
  • 49
    • 84922481787 scopus 로고    scopus 로고
    • Dissection of genetic factors associated with amyotrophic lateral sclerosis
    • Leblond, C.S., Kaneb, H.M., Dion, P.A., and Rouleau, G.A. 2014. Dissection of genetic factors associated with amyotrophic lateral sclerosis. Exp. Neurol. 262:91-101.
    • (2014) Exp. Neurol , vol.262 , pp. 91-101
    • Leblond, C.S.1    Kaneb, H.M.2    Dion, P.A.3    Rouleau, G.A.4
  • 50
    • 84155167265 scopus 로고    scopus 로고
    • Gains or losses: Molecular mechanisms of TDP43-mediated neurodegeneration
    • Lee, E.B., Lee, V.M., and Trojanowski, J.Q. 2012. Gains or losses: Molecular mechanisms of TDP43-mediated neurodegeneration. Nat. Rev. Neurosci. 13:38-50.
    • (2012) Nat. Rev. Neurosci , vol.13 , pp. 38-50
    • Lee, E.B.1    Lee, V.M.2    Trojanowski, J.Q.3
  • 52
    • 0037096354 scopus 로고    scopus 로고
    • Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease
    • Lino, M.M., Schneider, C., and Caroni, P. 2002. Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease. J. Neurosci. 22:4825-4832.
    • (2002) J. Neurosci , vol.22 , pp. 4825-4832
    • Lino, M.M.1    Schneider, C.2    Caroni, P.3
  • 54
    • 0029807527 scopus 로고    scopus 로고
    • Control of memory formation through regulated expression of a CaMKII transgene
    • Mayford, M., Bach, M.E., Huang, Y.Y., Wang, L., Hawkins, R.D., and Kandel, E.R. 1996. Control of memory formation through regulated expression of a CaMKII transgene. Science 274:1678-1683.
    • (1996) Science , vol.274 , pp. 1678-1683
    • Mayford, M.1    Bach, M.E.2    Huang, Y.Y.3    Wang, L.4    Hawkins, R.D.5    Kandel, E.R.6
  • 56
    • 0028264346 scopus 로고
    • Reactive astrocytes are widespread in the cortical gray matter of amyotrophic lateral sclerosis
    • Nagy, D., Kato, T., and Kushner, P.D. 1994. Reactive astrocytes are widespread in the cortical gray matter of amyotrophic lateral sclerosis. J. Neurosci. Res. 38:336-347.
    • (1994) J. Neurosci. Res , vol.38 , pp. 336-347
    • Nagy, D.1    Kato, T.2    Kushner, P.D.3
  • 59
    • 84892597157 scopus 로고    scopus 로고
    • Screening for frontal lobe and general cognitive impairment in patients with amyotrophic lateral sclerosis
    • Osborne, R.A., Sekhon, R., Johnston, W., and Kalra, S. 2014. Screening for frontal lobe and general cognitive impairment in patients with amyotrophic lateral sclerosis. J. Neurol. Sci. 336:191-196.
    • (2014) J. Neurol. Sci , vol.336 , pp. 191-196
    • Osborne, R.A.1    Sekhon, R.2    Johnston, W.3    Kalra, S.4
  • 60
    • 79952754297 scopus 로고    scopus 로고
    • Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G(9)(3)A transgenic ALS mice
    • Ozdinler, P.H., Benn, S., Yamamoto, T.H., Guzel, M., Brown, R.H. Jr., and Macklis, J.D. (2011). Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G(9)(3)A transgenic ALS mice. J. Neurosci. 31:4166-4177.
    • (2011) J. Neurosci , vol.31 , pp. 4166-4177
    • Ozdinler, P.H.1    Benn, S.2    Yamamoto, T.H.3    Guzel, M.4    Brown, R.H.5    Macklis, J.D.6
  • 61
    • 84897425769 scopus 로고    scopus 로고
    • Preclinical research: Make mouse studies work
    • Perrin, S. 2014. Preclinical research: Make mouse studies work. Nature 507:423-425.
    • (2014) Nature , vol.507 , pp. 423-425
    • Perrin, S.1
  • 62
    • 79951704433 scopus 로고    scopus 로고
    • Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease
    • Philips, T. and Robberecht, W. 2011. Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. Lancet Neurol. 10:253-263.
    • (2011) Lancet Neurol , vol.10 , pp. 253-263
    • Philips, T.1    Robberecht, W.2
  • 63
    • 84908176097 scopus 로고    scopus 로고
    • Glial cells in amyotrophic lateral sclerosis
    • Philips, T. and Rothstein, J.D. 2014. Glial cells in amyotrophic lateral sclerosis. Exp. Neurol. 262B:111-120.
    • (2014) Exp. Neurol , vol.262B , pp. 111-120
    • Philips, T.1    Rothstein, J.D.2
  • 65
    • 0035873076 scopus 로고    scopus 로고
    • Neuronspecific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment
    • Pramatarova, A., Laganiere, J., Roussel, J., Brisebois, K., and Rouleau, G.A. 2001. Neuronspecific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment. J. Neurosci. 21:3369-3374.
    • (2001) J. Neurosci , vol.21 , pp. 3369-3374
    • Pramatarova, A.1    Laganiere, J.2    Roussel, J.3    Brisebois, K.4    Rouleau, G.A.5
  • 66
    • 84901312256 scopus 로고    scopus 로고
    • Roles of vascular endothelial growth factor in amyotrophic lateral sclerosis
    • Pronto-Laborinho, A.C., Pinto, S., and de Carvalho, M. 2014. Roles of vascular endothelial growth factor in amyotrophic lateral sclerosis. BioMed Res. Int. 2014:947513.
    • (2014) BioMed Res. Int , vol.2014
    • Pronto-Laborinho, A.C.1    Pinto, S.2    de Carvalho, M.3
  • 69
    • 84893649256 scopus 로고    scopus 로고
    • State of play in amyotrophic lateral sclerosis genetics
    • Renton, A.E., Chio, A., and Traynor, B.J. 2014. State of play in amyotrophic lateral sclerosis genetics. Nat. Neurosci. 17:17-23.
    • (2014) Nat. Neurosci , vol.17 , pp. 17-23
    • Renton, A.E.1    Chio, A.2    Traynor, B.J.3
  • 71
    • 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. 1995. Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. U.S.A. 92:689-693.
    • (1995) Proc. Natl. Acad. Sci. U.S.A , 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
  • 72
    • 0027164824 scopus 로고
    • Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    • Rosen, D.R. 1993. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 364: 362.
    • (1993) Nature , vol.364 , pp. 362
    • Rosen, D.R.1
  • 73
    • 0026597010 scopus 로고
    • Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis
    • Rothstein, J.D., Martin, L.J., and Kuncl, R.W. 1992. Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. N. Engl. J. Med. 326:1464-1468.
    • (1992) N. Engl. J. Med , vol.326 , pp. 1464-1468
    • Rothstein, J.D.1    Martin, L.J.2    Kuncl, R.W.3
  • 76
    • 67349164383 scopus 로고    scopus 로고
    • A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice
    • Saxena, S., Cabuy, E., and Caroni, P. 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
  • 80
    • 77958022745 scopus 로고    scopus 로고
    • Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice
    • Shan, X., Chiang, P.M., Price, D.L., and Wong, P.C. 2010. Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. Proc. Natl. Acad. Sci. U.S.A. 107:16325-16330.
    • (2010) Proc. Natl. Acad. Sci. U.S.A , vol.107 , pp. 16325-16330
    • Shan, X.1    Chiang, P.M.2    Price, D.L.3    Wong, P.C.4
  • 86
    • 49249123273 scopus 로고    scopus 로고
    • Sex-specific behavioural effects of environmental enrichment in a transgenic mouse model of amyotrophic lateral sclerosis
    • Stam, N.C., Nithianantharajah, J., Howard, M.L., Atkin, J.D., Cheema, S.S., and Hannan, A.J. 2008. Sex-specific behavioural effects of environmental enrichment in a transgenic mouse model of amyotrophic lateral sclerosis. Eur. J. Neurosci. 28:717-723.
    • (2008) Eur. J. Neurosci , vol.28 , pp. 717-723
    • Stam, N.C.1    Nithianantharajah, J.2    Howard, M.L.3    Atkin, J.D.4    Cheema, S.S.5    Hannan, A.J.6
  • 87
    • 79955502687 scopus 로고    scopus 로고
    • Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS
    • Sun, Z., Diaz, Z., Fang, X., Hart, M.P., Chesi, A., Shorter, J., and Gitler, A.D. 2011. Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. PLoS Biology 9: e1000614.
    • (2011) PLoS Biology , vol.9
    • Sun, Z.1    Diaz, Z.2    Fang, X.3    Hart, M.P.4    Chesi, A.5    Shorter, J.6    Gitler, A.D.7
  • 88
    • 80052936462 scopus 로고    scopus 로고
    • Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments
    • Swarup, V., Phaneuf, D., Bareil, C., Robertson, J., Rouleau, G.A., Kriz, J., and Julien, J.P. 2011a. Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. Brain 134:2610-2626.
    • (2011) Brain , vol.134 , pp. 2610-2626
    • Swarup, V.1    Phaneuf, D.2    Bareil, C.3    Robertson, J.4    Rouleau, G.A.5    Kriz, J.6    Julien, J.P.7
  • 89
    • 84855515796 scopus 로고    scopus 로고
    • Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor kB-mediated pathogenic pathways
    • Swarup, V., Phaneuf, D., Dupre, N., Petri, S., Strong, M., Kriz, J., and Julien, J.P. 2011b. Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor kB-mediated pathogenic pathways. J. Exp. Med. 208:2429-2447.
    • (2011) J. Exp. Med , vol.208 , pp. 2429-2447
    • Swarup, V.1    Phaneuf, D.2    Dupre, N.3    Petri, S.4    Strong, M.5    Kriz, J.6    Julien, J.P.7
  • 90
    • 0028947317 scopus 로고
    • Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues
    • Taneja, K.L., McCurrach, M., Schalling, M., Housman, D., and Singer, R.H. 1995. Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues. J. Cell Biol. 128:995-1002.
    • (1995) J. Cell Biol , vol.128 , pp. 995-1002
    • Taneja, K.L.1    McCurrach, M.2    Schalling, M.3    Housman, D.4    Singer, R.H.5
  • 91
    • 84880217322 scopus 로고    scopus 로고
    • Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats
    • Tong, J., Huang, C., Bi, F., Wu, Q., Huang, B., Liu, X., Li, F., Zhou, H., and Xia, X.G. 2013. Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats. EMBO J. 32:1917-1926.
    • (2013) EMBO J , vol.32 , pp. 1917-1926
    • Tong, J.1    Huang, C.2    Bi, F.3    Wu, Q.4    Huang, B.5    Liu, X.6    Li, F.7    Zhou, H.8    Xia, X.G.9
  • 92
    • 77955395385 scopus 로고    scopus 로고
    • Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U
    • Tsai, K.J., Yang, C.H., Fang, Y.H., Cho, K.H., Chien, W.L., Wang, W.T., Wu, T.W., Lin, C.P., Fu, W.M., and Shen, C.K. 2010. Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U. J. Exp. Med. 207:1661-1673.
    • (2010) J. Exp. Med , vol.207 , pp. 1661-1673
    • Tsai, K.J.1    Yang, C.H.2    Fang, Y.H.3    Cho, K.H.4    Chien, W.L.5    Wang, W.T.6    Wu, T.W.7    Lin, C.P.8    Fu, W.M.9    Shen, C.K.10
  • 94
    • 77950528523 scopus 로고    scopus 로고
    • Dismutase-competent SOD1 mutant accumulation in myelinating Schwann cells is not detrimental to normal or transgenic ALS model mice
    • Turner, B.J., Ackerley, S., Davies, K.E., and Talbot, K. 2010. Dismutase-competent SOD1 mutant accumulation in myelinating Schwann cells is not detrimental to normal or transgenic ALS model mice. Hum. Mol. Genet. 19:815-824.
    • (2010) Hum. Mol. Genet , vol.19 , pp. 815-824
    • Turner, B.J.1    Ackerley, S.2    Davies, K.E.3    Talbot, K.4
  • 96
    • 22244460463 scopus 로고    scopus 로고
    • GluR2 deficiency accelerates motor neuron degeneration in a mouse model of amyotrophic lateral sclerosis
    • Van Damme, P., Braeken, D., Callewaert, G., Robberecht, W., and Van Den Bosch, L. 2005. GluR2 deficiency accelerates motor neuron degeneration in a mouse model of amyotrophic lateral sclerosis. J. Neuropathol. Exp. Neurol. 64:605-612.
    • (2005) J. Neuropathol. Exp. Neurol , vol.64 , pp. 605-612
    • Van Damme, P.1    Braeken, D.2    Callewaert, G.3    Robberecht, W.4    Van Den Bosch, L.5
  • 97
    • 0037067009 scopus 로고    scopus 로고
    • Minocycline delays disease onset and mortality in a transgenic model of ALS
    • Van Den Bosch, L., Tilkin, P., Lemmens, G., and Robberecht, W. 2002. Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport 13:1067-1070.
    • (2002) Neuroreport , vol.13 , pp. 1067-1070
    • Van Den Bosch, L.1    Tilkin, P.2    Lemmens, G.3    Robberecht, W.4
  • 100
    • 1842483843 scopus 로고    scopus 로고
    • Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein
    • Watts, G.D., Wymer, J., Kovach, M.J., Mehta, S.G., Mumm, S., Darvish, D., Pestronk, A., Whyte, M.P., and Kimonis, V.E. 2004. Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat. Genetics 36:377-381.
    • (2004) Nat. Genetics , vol.36 , pp. 377-381
    • Watts, G.D.1    Wymer, J.2    Kovach, M.J.3    Mehta, S.G.4    Mumm, S.5    Darvish, D.6    Pestronk, A.7    Whyte, M.P.8    Kimonis, V.E.9
  • 101
    • 73249152831 scopus 로고    scopus 로고
    • TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
    • Wegorzewska, I., Bell, S., Cairns, N.J., Miller, T.M., and Baloh, R.H. 2009. TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc. Natl. Acad. Sci. U.S.A. 106:18809-18814.
    • (2009) Proc. Natl. Acad. Sci. U.S.A , vol.106 , pp. 18809-18814
    • Wegorzewska, I.1    Bell, S.2    Cairns, N.J.3    Miller, T.M.4    Baloh, R.H.5
  • 102
    • 34447093377 scopus 로고    scopus 로고
    • Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice
    • Weihl, C.C., Miller, S.E., Hanson, P.I., and Pestronk, A. 2007. Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice. Hum. Mol. Genet. 16:919-928.
    • (2007) Hum. Mol. Genet , vol.16 , pp. 919-928
    • Weihl, C.C.1    Miller, S.E.2    Hanson, P.I.3    Pestronk, A.4
  • 104
    • 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. 1995. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14:1105-1116.
    • (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
  • 105
    • 84865028374 scopus 로고    scopus 로고
    • Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice
    • Wu, L.S., Cheng, W.C., and Shen, C.K. 2012. Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice. J. Biol. Chem. 287:27335-27344.
    • (2012) J. Biol. Chem , vol.287 , pp. 27335-27344
    • Wu, L.S.1    Cheng, W.C.2    Shen, C.K.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.