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Volumn 2, Issue , 2004, Pages 1143-1168

Models of Charcot-Marie-Tooth Disease

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EID: 84903010668     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1016/B978-012439510-7/50101-7     Document Type: Chapter
Times cited : (6)

References (209)
  • 1
    • 0032807346 scopus 로고    scopus 로고
    • Studies in transgenic mice indicate a loss of connexin32 function in X-linked Charcot-Marie-Tooth disease
    • Abel A., Bone L.J., Messing A., Scherer S.S., Fischbeck K.H. Studies in transgenic mice indicate a loss of connexin32 function in X-linked Charcot-Marie-Tooth disease. J Neuropathol Exp Neurol 1999, 58:702-710.
    • (1999) J Neuropathol Exp Neurol , vol.58 , pp. 702-710
    • Abel, A.1    Bone, L.J.2    Messing, A.3    Scherer, S.S.4    Fischbeck, K.H.5
  • 2
    • 0030994297 scopus 로고    scopus 로고
    • Heterozygous peripheral myelin protein 22-deficient mice are affected by a progressive demyelinating tomaculous neuropathy
    • Adlkofer K., Frei R., Neuberg D.H., Zielasek J., Toyka K.V., Suter U. Heterozygous peripheral myelin protein 22-deficient mice are affected by a progressive demyelinating tomaculous neuropathy. J Neurosci 1997, 17:4662-4671.
    • (1997) J Neurosci , vol.17 , pp. 4662-4671
    • Adlkofer, K.1    Frei, R.2    Neuberg, D.H.3    Zielasek, J.4    Toyka, K.V.5    Suter, U.6
  • 3
    • 0028784820 scopus 로고
    • Hypermyelination and demyelinating peripheral neuropathy in Pmp22-deficient mice
    • Adlkofer K., Martini R., Aguzzi A., Zielasek J., Toyka K.V., Suter U. Hypermyelination and demyelinating peripheral neuropathy in Pmp22-deficient mice. Nat Genet 1995, 11:274-280.
    • (1995) Nat Genet , vol.11 , pp. 274-280
    • Adlkofer, K.1    Martini, R.2    Aguzzi, A.3    Zielasek, J.4    Toyka, K.V.5    Suter, U.6
  • 4
    • 0030883723 scopus 로고    scopus 로고
    • Analysis of compound heterozygous mice reveals that the Trembler mutation can behave as a gain-of-function allele
    • Adlkofer K., Naef R., Suter U. Analysis of compound heterozygous mice reveals that the Trembler mutation can behave as a gain-of-function allele. J Neurosci Res 1997, 49:671-680.
    • (1997) J Neurosci Res , vol.49 , pp. 671-680
    • Adlkofer, K.1    Naef, R.2    Suter, U.3
  • 5
    • 0017593352 scopus 로고
    • Abnormal myelination in transplanted Trembler mouse Schwann cells
    • Aguayo A.J., Attiwell M., Trecarten J., Perkins S., Bray G.M. Abnormal myelination in transplanted Trembler mouse Schwann cells. Nature 1977, 265:73-75.
    • (1977) Nature , vol.265 , pp. 73-75
    • Aguayo, A.J.1    Attiwell, M.2    Trecarten, J.3    Perkins, S.4    Bray, G.M.5
  • 6
    • 0036703632 scopus 로고    scopus 로고
    • Connexin29 is uniquely distributed within myelinating glial cells of the central and peripheral nervous systems
    • Altevogt B.M., Kleopa K.A., Postma F.R., Scherer S.S., Paul D.L. Connexin29 is uniquely distributed within myelinating glial cells of the central and peripheral nervous systems. J Neurosci 2002, 22:6458-6470.
    • (2002) J Neurosci , vol.22 , pp. 6458-6470
    • Altevogt, B.M.1    Kleopa, K.A.2    Postma, F.R.3    Scherer, S.S.4    Paul, D.L.5
  • 7
    • 0030979840 scopus 로고    scopus 로고
    • Structural abnormalities and deficient maintenance of peripheral nerve myelin in mice lacking the gap junction protein connexin 32
    • Anzini P., Neuberg D.H., Schachner M., Nelles E., Willecke K., Zielasek J., Toyka K.V., Suter U., Martini R. Structural abnormalities and deficient maintenance of peripheral nerve myelin in mice lacking the gap junction protein connexin 32. J Neurosci 1997, 17:4545-4551.
    • (1997) J Neurosci , vol.17 , pp. 4545-4551
    • Anzini, P.1    Neuberg, D.H.2    Schachner, M.3    Nelles, E.4    Willecke, K.5    Zielasek, J.6    Toyka, K.V.7    Suter, U.8    Martini, R.9
  • 8
    • 0000094557 scopus 로고    scopus 로고
    • Myelinating Schwann cells determine the internodal localization of Kv1.1, Kv1.2, Kvbeta2, and Caspr
    • Arroyo E.J., Xu Y.T., Zhou L., Messing A., Peles E., Chiu S.Y., Scherer S.S. Myelinating Schwann cells determine the internodal localization of Kv1.1, Kv1.2, Kvbeta2, and Caspr. J Neurocytol 1999, 28:333-347.
    • (1999) J Neurocytol , vol.28 , pp. 333-347
    • Arroyo, E.J.1    Xu, Y.T.2    Zhou, L.3    Messing, A.4    Peles, E.5    Chiu, S.Y.6    Scherer, S.S.7
  • 9
    • 0037083127 scopus 로고    scopus 로고
    • Proliferation of Schwann cells and regulation of cyclin D1 expression in an animal model of Charcot-Marie-Tooth disease type 1A
    • Atanasoski S., Scherer S.S., Nave K.A., Suter U. Proliferation of Schwann cells and regulation of cyclin D1 expression in an animal model of Charcot-Marie-Tooth disease type 1A. J Neurosci Res 2002, 67:443-449.
    • (2002) J Neurosci Res , vol.67 , pp. 443-449
    • Atanasoski, S.1    Scherer, S.S.2    Nave, K.A.3    Suter, U.4
  • 10
    • 0035687780 scopus 로고    scopus 로고
    • Differential cyclin D1 requirements of proliferating Schwann cells during development and after injury
    • Atanasoski S., Shumas S., Dickson C., Scherer S.S., Suter U. Differential cyclin D1 requirements of proliferating Schwann cells during development and after injury. Mol Cell Neurosci 2001, 18:581-592.
    • (2001) Mol Cell Neurosci , vol.18 , pp. 581-592
    • Atanasoski, S.1    Shumas, S.2    Dickson, C.3    Scherer, S.S.4    Suter, U.5
  • 11
    • 0015785567 scopus 로고
    • Onion bulb neuropathy in the Trembler mouse: A model of hypertrophic interstitial neuropathy (Dejerine-Sottas). in man
    • Ayers M.M., Anderson R.M. Onion bulb neuropathy in the Trembler mouse: A model of hypertrophic interstitial neuropathy (Dejerine-Sottas). in man. Acta Neuropathol 1973, 25:54-70.
    • (1973) Acta Neuropathol , vol.25 , pp. 54-70
    • Ayers, M.M.1    Anderson, R.M.2
  • 12
    • 0016822082 scopus 로고
    • Development of onion bulb neuropathy in the Trembler mouse. Comparison with normal nerve maturation
    • Ayers M.M., Anderson R.M. Development of onion bulb neuropathy in the Trembler mouse. Comparison with normal nerve maturation. Acta Neuropathol 1975, 32:43-59.
    • (1975) Acta Neuropathol , vol.32 , pp. 43-59
    • Ayers, M.M.1    Anderson, R.M.2
  • 13
    • 0017107041 scopus 로고
    • Development of onion bulb neuropathy in the Trembler mouse. Morphometric study
    • Ayers M.M., Anderson R.M. Development of onion bulb neuropathy in the Trembler mouse. Morphometric study. Acta Neuropathol 1976, 36:137-152.
    • (1976) Acta Neuropathol , vol.36 , pp. 137-152
    • Ayers, M.M.1    Anderson, R.M.2
  • 14
    • 0032563605 scopus 로고    scopus 로고
    • Functional gap junctions in the schwann cell myelin sheath
    • Balice-Gordon R.J., Bone L.J., Scherer S.S. Functional gap junctions in the schwann cell myelin sheath. J Cell Biol 1998, 142:1095-1104.
    • (1998) J Cell Biol , vol.142 , pp. 1095-1104
    • Balice-Gordon, R.J.1    Bone, L.J.2    Scherer, S.S.3
  • 15
    • 0029921602 scopus 로고    scopus 로고
    • The myelin-associated glycoprotein of the peripheral nervous system in trembler mutants contains increased α2-3 sialic acid and galactose
    • Bartoszewics Z.P., Lauter C.J., Quarles R.H. The myelin-associated glycoprotein of the peripheral nervous system in trembler mutants contains increased α2-3 sialic acid and galactose. J Neurosci Res 1996, 43:587-593.
    • (1996) J Neurosci Res , vol.43 , pp. 587-593
    • Bartoszewics, Z.P.1    Lauter, C.J.2    Quarles, R.H.3
  • 16
    • 0026975041 scopus 로고
    • Expression of the PMP-22 gene in Trembler mutant mice: Comparison with the other myelin protein genes
    • Bascles L., Bonnet J., Garbay B. Expression of the PMP-22 gene in Trembler mutant mice: Comparison with the other myelin protein genes. Dev Neurosci 1992, 14:336-341.
    • (1992) Dev Neurosci , vol.14 , pp. 336-341
    • Bascles, L.1    Bonnet, J.2    Garbay, B.3
  • 17
    • 0034255676 scopus 로고    scopus 로고
    • Clinico-electrophysiological correlation of extensor digitorum brevis muscle atrophy in children with charcot-marie-tooth disease 1A duplication
    • Berciano J., Garcia A., Calleja J., Combarros O. Clinico-electrophysiological correlation of extensor digitorum brevis muscle atrophy in children with charcot-marie-tooth disease 1A duplication. Neuromuscul Disord 2000, 10:419-424.
    • (2000) Neuromuscul Disord , vol.10 , pp. 419-424
    • Berciano, J.1    Garcia, A.2    Calleja, J.3    Combarros, O.4
  • 18
    • 0036524611 scopus 로고    scopus 로고
    • Molecular cell biology of Charcot-Marie-Tooth disease
    • Berger P., Young P., Suter U. Molecular cell biology of Charcot-Marie-Tooth disease. Neurogenetics 2002, 4:1-15.
    • (2002) Neurogenetics , vol.4 , pp. 1-15
    • Berger, P.1    Young, P.2    Suter, U.3
  • 20
    • 0033554343 scopus 로고    scopus 로고
    • Historical perspective of defining Charcot-Marie-Tooth type 1B
    • Bird T.D. Historical perspective of defining Charcot-Marie-Tooth type 1B. Ann N Y Acad Sci 1999, 883:6-13.
    • (1999) Ann N Y Acad Sci , vol.883 , pp. 6-13
    • Bird, T.D.1
  • 22
    • 0028857595 scopus 로고
    • Cerebroside formation in the peripheral nervous system of normal and Trembler mice
    • Boiron-Sargueil F., Heape A., Garbay B., Cassagne C. Cerebroside formation in the peripheral nervous system of normal and Trembler mice. Neurosci Lett 1995, 186:21-24.
    • (1995) Neurosci Lett , vol.186 , pp. 21-24
    • Boiron-Sargueil, F.1    Heape, A.2    Garbay, B.3    Cassagne, C.4
  • 23
    • 0040520433 scopus 로고
    • Neurological actions caused by the mutant gene "Trembler" in the house mouse (Mus musculus L.): An investigation
    • Braverman I.M. Neurological actions caused by the mutant gene "Trembler" in the house mouse (Mus musculus L.): An investigation. J Neuropathol Exp Neurol 1953, 12:64-72.
    • (1953) J Neuropathol Exp Neurol , vol.12 , pp. 64-72
    • Braverman, I.M.1
  • 26
    • 0027293659 scopus 로고
    • Hereditary motor-sensory neuropathy and movement disorders
    • Cardoso F.E., Jankovic J. Hereditary motor-sensory neuropathy and movement disorders. Muscle Nerve 1993, 16:904-910.
    • (1993) Muscle Nerve , vol.16 , pp. 904-910
    • Cardoso, F.E.1    Jankovic, J.2
  • 28
    • 0031721031 scopus 로고    scopus 로고
    • Hereditary neuropathy with liability to pressure palsies: A patient's point mutation in a mouse model
    • Chance P.F., Dyck P.J. Hereditary neuropathy with liability to pressure palsies: A patient's point mutation in a mouse model. Neurology 1998, 51:664-665.
    • (1998) Neurology , vol.51 , pp. 664-665
    • Chance, P.F.1    Dyck, P.J.2
  • 31
    • 0025253083 scopus 로고
    • Protein zero of peripheral nerve myelin: Biosynthesis, membrane insertion, and evidence for homotypic interaction
    • D'Urso D., Brophy P.J., Staugaitis S.M., Gillespie C.S., Frey A.B., Stempak J.G., Colman D.R. Protein zero of peripheral nerve myelin: Biosynthesis, membrane insertion, and evidence for homotypic interaction. Neuron 1990, 4:449-460.
    • (1990) Neuron , vol.4 , pp. 449-460
    • D'Urso, D.1    Brophy, P.J.2    Staugaitis, S.M.3    Gillespie, C.S.4    Frey, A.B.5    Stempak, J.G.6    Colman, D.R.7
  • 32
    • 0031972929 scopus 로고    scopus 로고
    • Overloaded endoplasmic reticulum-Golgi compartments, a possible pathomechanism of peripheral neuropathies caused by mutations of the peripheral myelin protein PMP22
    • D'Urso D., Prior R., Greiner-Petter R., Gabreels-Festen A.A., Muller H.W. Overloaded endoplasmic reticulum-Golgi compartments, a possible pathomechanism of peripheral neuropathies caused by mutations of the peripheral myelin protein PMP22. J Neurosci 1998, 18:731-740.
    • (1998) J Neurosci , vol.18 , pp. 731-740
    • D'Urso, D.1    Prior, R.2    Greiner-Petter, R.3    Gabreels-Festen, A.A.4    Muller, H.W.5
  • 33
    • 0030887142 scopus 로고    scopus 로고
    • Studies on the effects of altered PMP22 expression during myelination in vitro
    • D'Urso D., Schmalenbach C., Zoidl G., Prior R., Muller H.W. Studies on the effects of altered PMP22 expression during myelination in vitro. J Neurosci Res 1997, 48:31-42.
    • (1997) J Neurosci Res , vol.48 , pp. 31-42
    • D'Urso, D.1    Schmalenbach, C.2    Zoidl, G.3    Prior, R.4    Muller, H.W.5
  • 36
    • 0028200642 scopus 로고
    • SR13/PMP-22 expression in rat nervous system, in PC12 cells, and C6 glial cell lines
    • De Leon M., Nahin R.L., Mendoza M.E., Ruda M.A. SR13/PMP-22 expression in rat nervous system, in PC12 cells, and C6 glial cell lines. J Neurosci Res 1994, 38:167-181.
    • (1994) J Neurosci Res , vol.38 , pp. 167-181
    • De Leon, M.1    Nahin, R.L.2    Mendoza, M.E.3    Ruda, M.A.4
  • 38
    • 0025438055 scopus 로고
    • Altered slow axonal transport and regeneration in a myelin-deficient mutant mouse: The trembler as an in vivo model for Schwann cell-axon interactions
    • de Waegh S., Brady S.T. Altered slow axonal transport and regeneration in a myelin-deficient mutant mouse: The trembler as an in vivo model for Schwann cell-axon interactions. J Neurosci 1990, 10:1855-1865.
    • (1990) J Neurosci , vol.10 , pp. 1855-1865
    • de Waegh, S.1    Brady, S.T.2
  • 39
    • 0025949638 scopus 로고
    • Local control of axonal properties by Schwann cells: Neurofilaments and axonal transport in homologous and heterologous nerve grafts
    • de Waegh S.M., Brady S.T. Local control of axonal properties by Schwann cells: Neurofilaments and axonal transport in homologous and heterologous nerve grafts. J Neurosci Res 1991, 30:201-212.
    • (1991) J Neurosci Res , vol.30 , pp. 201-212
    • de Waegh, S.M.1    Brady, S.T.2
  • 40
    • 0026580004 scopus 로고
    • Local modulation of neurofilament phosphorylation, axonal caliber, and slow axonal transport by myelinating Schwann cells
    • de Waegh S.M., Lee V.M., Brady S.T. Local modulation of neurofilament phosphorylation, axonal caliber, and slow axonal transport by myelinating Schwann cells. Cell 1992, 68:451-463.
    • (1992) Cell , vol.68 , pp. 451-463
    • de Waegh, S.M.1    Lee, V.M.2    Brady, S.T.3
  • 42
    • 0024457455 scopus 로고
    • Longitudinal study of neuropathic deficits and nerve conduction abnormalities in hereditary motor and sensory neuropathy type 1
    • Dyck P.J., Karnes J.L., Lambert E.H. Longitudinal study of neuropathic deficits and nerve conduction abnormalities in hereditary motor and sensory neuropathy type 1. Neurology 1989, 39:1302-1308.
    • (1989) Neurology , vol.39 , pp. 1302-1308
    • Dyck, P.J.1    Karnes, J.L.2    Lambert, E.H.3
  • 43
    • 65749318026 scopus 로고
    • Two new mutants, "Trembler" and "Reeler" with neurological actions in the house mouse
    • Falconer D.S. Two new mutants, "Trembler" and "Reeler" with neurological actions in the house mouse. Genetics 1951, 50:192-201.
    • (1951) Genetics , vol.50 , pp. 192-201
    • Falconer, D.S.1
  • 44
    • 0025194356 scopus 로고
    • Role of myelin Po protein as a homophilic adhesion molecule
    • Filbin M., Walsh F., Trapp B., Pizzey J., Tennekoon G. Role of myelin Po protein as a homophilic adhesion molecule. Nature 1990, 344:871-872.
    • (1990) Nature , vol.344 , pp. 871-872
    • Filbin, M.1    Walsh, F.2    Trapp, B.3    Pizzey, J.4    Tennekoon, G.5
  • 46
    • 0345211489 scopus 로고    scopus 로고
    • Loss of distal axons and sensory Merkel cells and features indicative of muscle denervation in hindlimbs of P0-deficient mice
    • Frei R., Motzing S., Kinkelin I., Schachner M., Koltzenburg M., Martini R. Loss of distal axons and sensory Merkel cells and features indicative of muscle denervation in hindlimbs of P0-deficient mice. J Neurosci 1999, 19:6058-6067.
    • (1999) J Neurosci , vol.19 , pp. 6058-6067
    • Frei, R.1    Motzing, S.2    Kinkelin, I.3    Schachner, M.4    Koltzenburg, M.5    Martini, R.6
  • 48
    • 0028884642 scopus 로고
    • Expression of the exon 1A-containing PMP22 transcript is altered in the trembler mouse
    • Garbay B., Boiron-Sargueil F., Cassagne C. Expression of the exon 1A-containing PMP22 transcript is altered in the trembler mouse. Neurosci Lett 1995, 198:157-160.
    • (1995) Neurosci Lett , vol.198 , pp. 157-160
    • Garbay, B.1    Boiron-Sargueil, F.2    Cassagne, C.3
  • 49
    • 0031718046 scopus 로고    scopus 로고
    • Regulation of oleoyl-CoA synthesis in the peripheral nervous system: Demonstration of a link with myelin synthesis
    • Garbay B., Boiron-Sargueil F., Shy M., Chbihi T., Jiang H., Kamholz J., Cassagne C. Regulation of oleoyl-CoA synthesis in the peripheral nervous system: Demonstration of a link with myelin synthesis. J Neurochem 1998, 71:1719-1726.
    • (1998) J Neurochem , vol.71 , pp. 1719-1726
    • Garbay, B.1    Boiron-Sargueil, F.2    Shy, M.3    Chbihi, T.4    Jiang, H.5    Kamholz, J.6    Cassagne, C.7
  • 50
    • 0026616907 scopus 로고
    • P0 protein in normal, trembler heterozygous/homozygous mice during active PNS myelination
    • Garbay B., Bonnet J. P0 protein in normal, trembler heterozygous/homozygous mice during active PNS myelination. Neuroreport 1992, 3:594-596.
    • (1992) Neuroreport , vol.3 , pp. 594-596
    • Garbay, B.1    Bonnet, J.2
  • 51
    • 0028152855 scopus 로고
    • Expression of the ceramide galactosyltransferase gene during myelination of the mouse nervous system. Comparison with the genes encoding myelin basic proteins, choline kinase and CTP:phosphocholine cytidyltransferase
    • Garbay B., Cassagne C. Expression of the ceramide galactosyltransferase gene during myelination of the mouse nervous system. Comparison with the genes encoding myelin basic proteins, choline kinase and CTP:phosphocholine cytidyltransferase. Dev Brain Res 1994, 83:119-124.
    • (1994) Dev Brain Res , vol.83 , pp. 119-124
    • Garbay, B.1    Cassagne, C.2
  • 53
    • 0026615047 scopus 로고
    • Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons
    • Giese K.P., Martini R., Lemke G., Soriano P., Schachner M. Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons. Cell 1992, 71:565-576.
    • (1992) Cell , vol.71 , pp. 565-576
    • Giese, K.P.1    Martini, R.2    Lemke, G.3    Soriano, P.4    Schachner, M.5
  • 54
    • 0028204901 scopus 로고
    • Periaxin, a novel protein of myelinating Schwann cells with a possible role in axonal ensheathment
    • Gillespie C.S., Sherman D.L., Blair G.E., Brophy P.J. Periaxin, a novel protein of myelinating Schwann cells with a possible role in axonal ensheathment. Neuron 1994, 12:497-508.
    • (1994) Neuron , vol.12 , pp. 497-508
    • Gillespie, C.S.1    Sherman, D.L.2    Blair, G.E.3    Brophy, P.J.4
  • 56
    • 0032559544 scopus 로고    scopus 로고
    • Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease
    • Gow A., Southwood C.M., Lazzarini R.A. Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J Cell Biol 1998, 140:925-934.
    • (1998) J Cell Biol , vol.140 , pp. 925-934
    • Gow, A.1    Southwood, C.M.2    Lazzarini, R.A.3
  • 57
    • 0015609956 scopus 로고
    • Protein composition of myelin of the peripheral nervous system
    • Greenfield S., Brostoff S., Eylar E.H., Morell P. Protein composition of myelin of the peripheral nervous system. J. Neurochem 1973, 20:1207-1216.
    • (1973) J. Neurochem , vol.20 , pp. 1207-1216
    • Greenfield, S.1    Brostoff, S.2    Eylar, E.H.3    Morell, P.4
  • 60
    • 0032871477 scopus 로고    scopus 로고
    • P0 and PMP22 mark a multipotent neural crest-derived cell type that displays community effects in response to TGF-beta family factors
    • Hagedorn L., Suter U., Sommer L. P0 and PMP22 mark a multipotent neural crest-derived cell type that displays community effects in response to TGF-beta family factors. Development 1999, 126:3781-3794.
    • (1999) Development , vol.126 , pp. 3781-3794
    • Hagedorn, L.1    Suter, U.2    Sommer, L.3
  • 62
    • 0030560999 scopus 로고    scopus 로고
    • Pathways of incorporation of fatty acid into glycerolipids of the murine peripheral nervous system in vivo: Alterations in the dysmyelinating mutant trembler mouse
    • Heape A.M., Bessoule J.J., Boiron-Sargueil F., Cassagne C. Pathways of incorporation of fatty acid into glycerolipids of the murine peripheral nervous system in vivo: Alterations in the dysmyelinating mutant trembler mouse. Neurochem Int 1996, 29:607-622.
    • (1996) Neurochem Int , vol.29 , pp. 607-622
    • Heape, A.M.1    Bessoule, J.J.2    Boiron-Sargueil, F.3    Cassagne, C.4
  • 63
    • 0029154336 scopus 로고
    • Sphingolipid metabolic disorders in Trembler mouse peripheral nerves in vivo result from an abnormal substrate supply
    • Heape A.M., Bessoule J.-J., Boiron-Sargueil F., Garbay B., Cassagne C. Sphingolipid metabolic disorders in Trembler mouse peripheral nerves in vivo result from an abnormal substrate supply. J Neurochem 1995, 65:1665-1673.
    • (1995) J Neurochem , vol.65 , pp. 1665-1673
    • Heape, A.M.1    Bessoule, J.-J.2    Boiron-Sargueil, F.3    Garbay, B.4    Cassagne, C.5
  • 64
    • 0025922687 scopus 로고
    • Distribution of P0 protein and the myelin-associated glycoprotein in peripheral nerves from Trembler mice
    • Heath J.W., Inuzuka T., Quarles R.H., Trapp B.D. Distribution of P0 protein and the myelin-associated glycoprotein in peripheral nerves from Trembler mice. J Neurocytol 1991, 20:439-449.
    • (1991) J Neurocytol , vol.20 , pp. 439-449
    • Heath, J.W.1    Inuzuka, T.2    Quarles, R.H.3    Trapp, B.D.4
  • 65
    • 0021080193 scopus 로고
    • Comparison of trembler and trembler-J phenotypes: Varying severity of peripheral hypomyelination
    • Henry E.W., Cowen J.S., Sidman R.L. Comparison of trembler and trembler-J phenotypes: Varying severity of peripheral hypomyelination. J Neuropathol Exp Neurol 1983, 42:688-706.
    • (1983) J Neuropathol Exp Neurol , vol.42 , pp. 688-706
    • Henry, E.W.1    Cowen, J.S.2    Sidman, R.L.3
  • 66
    • 0026395971 scopus 로고
    • The mouse mutation claw paw: Forelimb deformity and delayed myelination throughout the peripheral nervous system
    • Henry E.W., Eicher E.M., Sidman R.L. The mouse mutation claw paw: Forelimb deformity and delayed myelination throughout the peripheral nervous system. J Hered 1991, 82:287-294.
    • (1991) J Hered , vol.82 , pp. 287-294
    • Henry, E.W.1    Eicher, E.M.2    Sidman, R.L.3
  • 67
    • 0021079136 scopus 로고
    • The murine mutation trembler-J: Proof of semi-dominant expression by the use of the linked vestigial tail marker
    • Henry E.W., Sidman R.L. The murine mutation trembler-J: Proof of semi-dominant expression by the use of the linked vestigial tail marker. J Neurogen 1983, 1:39-52.
    • (1983) J Neurogen , vol.1 , pp. 39-52
    • Henry, E.W.1    Sidman, R.L.2
  • 68
    • 0023794823 scopus 로고
    • Long lives for homozygous trembler mutant mice despite virtual absence of peripheral myelin
    • Henry E.W., Sidman R.L. Long lives for homozygous trembler mutant mice despite virtual absence of peripheral myelin. Science 1988, 241:344-346.
    • (1988) Science , vol.241 , pp. 344-346
    • Henry, E.W.1    Sidman, R.L.2
  • 71
    • 0035577854 scopus 로고    scopus 로고
    • Hedgehog signaling in animal development: Paradigms and principles
    • Ingham P.W., McMahon A.P. Hedgehog signaling in animal development: Paradigms and principles. Genes Dev 2001, 15:3059-3087.
    • (2001) Genes Dev , vol.15 , pp. 3059-3087
    • Ingham, P.W.1    McMahon, A.P.2
  • 72
    • 0034830932 scopus 로고    scopus 로고
    • The 1.4-Mb CMT1A duplication/HNPP deletion genomic region reveals unique genome architectural features and provides insights into the recent evolution of new genes
    • Inoue K., Dewar K., Katsanis N., Reiter L.T., Lander E.S., Devon K.L., Wyman D.W., Lupski J.R., Birren B. The 1.4-Mb CMT1A duplication/HNPP deletion genomic region reveals unique genome architectural features and provides insights into the recent evolution of new genes. Genome Res 2001, 11:1018-1033.
    • (2001) Genome Res , vol.11 , pp. 1018-1033
    • Inoue, K.1    Dewar, K.2    Katsanis, N.3    Reiter, L.T.4    Lander, E.S.5    Devon, K.L.6    Wyman, D.W.7    Lupski, J.R.8    Birren, B.9
  • 73
    • 0036894042 scopus 로고    scopus 로고
    • Congenital hypomyelinating neuropathy, central dysmyelination, and Waardenburg-Hirschsprung disease: Phenotypes linked by SOX10 mutation
    • Inoue K., Shilo K., Boerkoel C.F., Crowe C., Sawady J., Lupski J.R., Agamanolis D.P. Congenital hypomyelinating neuropathy, central dysmyelination, and Waardenburg-Hirschsprung disease: Phenotypes linked by SOX10 mutation. Ann Neurol 2002, 52:836-842.
    • (2002) Ann Neurol , vol.52 , pp. 836-842
    • Inoue, K.1    Shilo, K.2    Boerkoel, C.F.3    Crowe, C.4    Sawady, J.5    Lupski, J.R.6    Agamanolis, D.P.7
  • 74
    • 0032833425 scopus 로고    scopus 로고
    • Myelin deficiencies in both the central and the peripheral nervous systems associated with a SOX 10 mutation
    • Inoue K., Tanabe Y., Lupski J.R. Myelin deficiencies in both the central and the peripheral nervous systems associated with a SOX 10 mutation. Ann Neurol 1999, 46:313-318.
    • (1999) Ann Neurol , vol.46 , pp. 313-318
    • Inoue, K.1    Tanabe, Y.2    Lupski, J.R.3
  • 75
    • 0021986367 scopus 로고
    • Myelin-associated glycoprotein and other proteins in Trembler mice
    • Inuzuka T., Quarles R.H., Heath J., Trapp B.D. Myelin-associated glycoprotein and other proteins in Trembler mice. J Neurochem 1985, 44:793-797.
    • (1985) J Neurochem , vol.44 , pp. 793-797
    • Inuzuka, T.1    Quarles, R.H.2    Heath, J.3    Trapp, B.D.4
  • 79
    • 0031683605 scopus 로고    scopus 로고
    • Transgenic mice in the study of ALS: The role of neurofilaments
    • Julien J.P., Couillard-Despres S., Meier J. Transgenic mice in the study of ALS: The role of neurofilaments. Brain Pathol 1998, 8:759-769.
    • (1998) Brain Pathol , vol.8 , pp. 759-769
    • Julien, J.P.1    Couillard-Despres, S.2    Meier, J.3
  • 80
    • 0028143554 scopus 로고
    • Modulation of the axonal microtubule cytoskeleton by myelinating Schwann cells
    • Kirkpatrick L.L., Brady S.T. Modulation of the axonal microtubule cytoskeleton by myelinating Schwann cells. J Neurosci 1994, 14:7440-7450.
    • (1994) J Neurosci , vol.14 , pp. 7440-7450
    • Kirkpatrick, L.L.1    Brady, S.T.2
  • 81
    • 0017135073 scopus 로고
    • X-ray diffraction study of myelin structure in immature and mutant mice
    • Kirschner D.A., Sidman R.L. X-ray diffraction study of myelin structure in immature and mutant mice. Biochim Biophys Acta 1976, 448:73-87.
    • (1976) Biochim Biophys Acta , vol.448 , pp. 73-87
    • Kirschner, D.A.1    Sidman, R.L.2
  • 83
  • 84
    • 0036605376 scopus 로고    scopus 로고
    • Cellular mechanisms of connexin32 mutations associated with CNS manifestations
    • Kleopa K.A., Yum S.W., Scherer S.S. Cellular mechanisms of connexin32 mutations associated with CNS manifestations. J Neurosci Res 2002, 68:522-534.
    • (2002) J Neurosci Res , vol.68 , pp. 522-534
    • Kleopa, K.A.1    Yum, S.W.2    Scherer, S.S.3
  • 86
    • 0036499098 scopus 로고    scopus 로고
    • Macrophage-related demyclination in peripheral nerves of mice deficient in the gap junction protein connexin 32
    • Kobsar I., Maurer M., Ott T., Martini R. Macrophage-related demyclination in peripheral nerves of mice deficient in the gap junction protein connexin 32. Neurosci Lett 2002, 320:17-20.
    • (2002) Neurosci Lett , vol.320 , pp. 17-20
    • Kobsar, I.1    Maurer, M.2    Ott, T.3    Martini, R.4
  • 89
    • 0030824001 scopus 로고    scopus 로고
    • Morphogenesis of the node of Ranvier: Co-clusters of ankyrin and ankyrin-binding integral proteins define early developmental intermediates
    • Lambert S., Davis J.Q., Bennett V. Morphogenesis of the node of Ranvier: Co-clusters of ankyrin and ankyrin-binding integral proteins define early developmental intermediates. J Neurosci 1997, 17:7025-7036.
    • (1997) J Neurosci , vol.17 , pp. 7025-7036
    • Lambert, S.1    Davis, J.Q.2    Bennett, V.3
  • 90
    • 0028116467 scopus 로고
    • A mutant neurofilament subunit causes massive, selective motor neuron death: Implications for the pathogenesis of human motor neuron disease
    • Lee M.K., Marszalek J.R., Cleveland D.W. A mutant neurofilament subunit causes massive, selective motor neuron death: Implications for the pathogenesis of human motor neuron disease. Neuron 1994, 13:975-988.
    • (1994) Neuron , vol.13 , pp. 975-988
    • Lee, M.K.1    Marszalek, J.R.2    Cleveland, D.W.3
  • 91
    • 0021849731 scopus 로고
    • Isolation and sequence of a cDNA encoding the major structural protein of peripheral nerve myelin
    • Lemke G., Axel R. Isolation and sequence of a cDNA encoding the major structural protein of peripheral nerve myelin. Cell 1985, 40:501-508.
    • (1985) Cell , vol.40 , pp. 501-508
    • Lemke, G.1    Axel, R.2
  • 92
    • 0017151543 scopus 로고
    • Hereditary hypertrophic neuropathy in the trembler mouse: Part 1: Histopathological studies: Light microscopy
    • Low P.A. Hereditary hypertrophic neuropathy in the trembler mouse: Part 1: Histopathological studies: Light microscopy. J Neurol Sci 1976, 30:327-341.
    • (1976) J Neurol Sci , vol.30 , pp. 327-341
    • Low, P.A.1
  • 93
    • 0017194584 scopus 로고
    • Hereditary hypertrophic neuropathy in the trembler mouse: Part 2: Histopathological studies: Electron microscopy
    • Low P.A. Hereditary hypertrophic neuropathy in the trembler mouse: Part 2: Histopathological studies: Electron microscopy. J Neurol Sci 1976, 30:343-368.
    • (1976) J Neurol Sci , vol.30 , pp. 343-368
    • Low, P.A.1
  • 94
    • 0017332395 scopus 로고
    • The evolution of 'onion bulbs' in the hereditary hypertrophic neuropathy of the Trembler mouse
    • Low P.A. The evolution of 'onion bulbs' in the hereditary hypertrophic neuropathy of the Trembler mouse. Neuropath Appl Neurobiol 1977, 3:81-92.
    • (1977) Neuropath Appl Neurobiol , vol.3 , pp. 81-92
    • Low, P.A.1
  • 95
    • 0016784725 scopus 로고
    • Hereditary demyelinating neuropathy in the trembler mouse
    • Low P.A., McLeod J.G. Hereditary demyelinating neuropathy in the trembler mouse. J Neurol Sci 1975, 26:565-575.
    • (1975) J Neurol Sci , vol.26 , pp. 565-575
    • Low, P.A.1    McLeod, J.G.2
  • 96
    • 0000907007 scopus 로고    scopus 로고
    • Charcot-Marie-Tooth peripheral neuropathies and related disorders
    • McGraw-Hill, Amsterdam, C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle, B. Childs, K.W. Kinzler, B. Vogelstein (Eds.)
    • Lupski J., Garcia C. Charcot-Marie-Tooth peripheral neuropathies and related disorders. The Metabolic & Molecular Bases of Inherited Disease 2001, Vol. 3:5759-5788. McGraw-Hill, Amsterdam. C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle, B. Childs, K.W. Kinzler, B. Vogelstein (Eds.).
    • (2001) The Metabolic & Molecular Bases of Inherited Disease , vol.3 , pp. 5759-5788
    • Lupski, J.1    Garcia, C.2
  • 98
    • 0036282683 scopus 로고    scopus 로고
    • Identification of the regulatory region of the peripheral myelin protein 22 (PMP22). gene that directs temporal and spatial expression in development and regeneration of peripheral nerves
    • Maier M., Berger P., Nave K.A., Suter U. Identification of the regulatory region of the peripheral myelin protein 22 (PMP22). gene that directs temporal and spatial expression in development and regeneration of peripheral nerves. Mol Cell Neurosci 2002, 20:93-109.
    • (2002) Mol Cell Neurosci , vol.20 , pp. 93-109
    • Maier, M.1    Berger, P.2    Nave, K.A.3    Suter, U.4
  • 99
    • 0036247602 scopus 로고    scopus 로고
    • Understanding Schwann cell-neurone interactions: The key to Charcot-Marie-Tooth disease?
    • Maier M., Berger P., Suter U. Understanding Schwann cell-neurone interactions: The key to Charcot-Marie-Tooth disease?. J Anat 2002, 200:357-366.
    • (2002) J Anat , vol.200 , pp. 357-366
    • Maier, M.1    Berger, P.2    Suter, U.3
  • 100
    • 0031842421 scopus 로고    scopus 로고
    • Charcot-Marie-Tooth disease type 2 associated with mutation of the myelin protein zero gene
    • Marrosu M.G., Vaccargiu S., Marrosu G., Vannelli A., Cianchetti C., Muntoni F. Charcot-Marie-Tooth disease type 2 associated with mutation of the myelin protein zero gene. Neurology 1998, 50:1397-1401.
    • (1998) Neurology , vol.50 , pp. 1397-1401
    • Marrosu, M.G.1    Vaccargiu, S.2    Marrosu, G.3    Vannelli, A.4    Cianchetti, C.5    Muntoni, F.6
  • 101
    • 0030696172 scopus 로고    scopus 로고
    • Animal models for inherited peripheral neuropathies
    • Martini R. Animal models for inherited peripheral neuropathies. J Anat 1997, 191:321-336.
    • (1997) J Anat , vol.191 , pp. 321-336
    • Martini, R.1
  • 102
    • 0034254710 scopus 로고    scopus 로고
    • Animal models for inherited peripheral neuropathies: Chances to find treatment strategies?
    • Martini R. Animal models for inherited peripheral neuropathies: Chances to find treatment strategies?. J Neurosci Res 2000, 61:244-250.
    • (2000) J Neurosci Res , vol.61 , pp. 244-250
    • Martini, R.1
  • 103
    • 0035078628 scopus 로고    scopus 로고
    • The effect of myelinating Schwann cells on axons
    • Martini R. The effect of myelinating Schwann cells on axons. Muscle Nerve 2001, 24:456-466.
    • (2001) Muscle Nerve , vol.24 , pp. 456-466
    • Martini, R.1
  • 104
    • 0028824925 scopus 로고
    • Protein zero (P0)-deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies
    • Martini R., Zielasek J., Toyka K.V., Giese K.P., Schachner M. Protein zero (P0)-deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies. Nat. Genet. 1995, 11:281-286.
    • (1995) Nat. Genet. , vol.11 , pp. 281-286
    • Martini, R.1    Zielasek, J.2    Toyka, K.V.3    Giese, K.P.4    Schachner, M.5
  • 105
    • 0036252837 scopus 로고    scopus 로고
    • Role of immune cells in animal models for inherited neuropathies: Facts and visions
    • Maurer M., Kobsar I., Berghoff M., Schmid C.D., Carenini S., Martini R. Role of immune cells in animal models for inherited neuropathies: Facts and visions. J Anat 2002, 200:405-414.
    • (2002) J Anat , vol.200 , pp. 405-414
    • Maurer, M.1    Kobsar, I.2    Berghoff, M.3    Schmid, C.D.4    Carenini, S.5    Martini, R.6
  • 106
    • 0034948117 scopus 로고    scopus 로고
    • Bone marrow transfer from wild-type mice reverts the beneficial effect of genetically mediated immune deficiency in myelin mutants
    • Maurer M., Schmid C.D., Bootz F., Zielasek J., Toyka K.V., Oehen S., Martini R. Bone marrow transfer from wild-type mice reverts the beneficial effect of genetically mediated immune deficiency in myelin mutants. Mol Cell Neurosci 2001, 17:1094-1101.
    • (2001) Mol Cell Neurosci , vol.17 , pp. 1094-1101
    • Maurer, M.1    Schmid, C.D.2    Bootz, F.3    Zielasek, J.4    Toyka, K.V.5    Oehen, S.6    Martini, R.7
  • 109
    • 0035201307 scopus 로고    scopus 로고
    • The structure and function of nuclear lamins: Implications for disease
    • Moir R.D., Spann T.P. The structure and function of nuclear lamins: Implications for disease. Cell Mol Life Sci 2001, 58:1748-1757.
    • (2001) Cell Mol Life Sci , vol.58 , pp. 1748-1757
    • Moir, R.D.1    Spann, T.P.2
  • 110
    • 0036662882 scopus 로고    scopus 로고
    • Association of the kinesin superfamily motor protein KIF1Balpha with postsynaptic density-95 (PSD-95), synapse-associated protein-97, and synaptic scaffolding molecule PSD-95/discs large/zona occludens-1 proteins
    • Mok H., Shin H., Kim S., Lee J.R., Yoon J., Kim E. Association of the kinesin superfamily motor protein KIF1Balpha with postsynaptic density-95 (PSD-95), synapse-associated protein-97, and synaptic scaffolding molecule PSD-95/discs large/zona occludens-1 proteins. J Neurosci 2002, 22:5253-5258.
    • (2002) J Neurosci , vol.22 , pp. 5253-5258
    • Mok, H.1    Shin, H.2    Kim, S.3    Lee, J.R.4    Yoon, J.5    Kim, E.6
  • 112
    • 0030900850 scopus 로고    scopus 로고
    • Aberrant protein trafficking in Trembler suggests a disease mechanism for hereditary human peripheral neuropathies
    • Naef R., Adlkofer K., Lescher B., Suter U. Aberrant protein trafficking in Trembler suggests a disease mechanism for hereditary human peripheral neuropathies. Mol Cell Neurosci 1997, 9:13-25.
    • (1997) Mol Cell Neurosci , vol.9 , pp. 13-25
    • Naef, R.1    Adlkofer, K.2    Lescher, B.3    Suter, U.4
  • 113
    • 0037173037 scopus 로고    scopus 로고
    • Deciphering peripheral nerve myelination by using Schwann cell expression profiling
    • Nagarajan R., Le N., Mahoney H., Araki T., Milbrandt J. Deciphering peripheral nerve myelination by using Schwann cell expression profiling. Proc Natl Acad Sci U S A 2002, 99:8998-9003.
    • (2002) Proc Natl Acad Sci U S A , vol.99 , pp. 8998-9003
    • Nagarajan, R.1    Le, N.2    Mahoney, H.3    Araki, T.4    Milbrandt, J.5
  • 114
    • 0034981923 scopus 로고    scopus 로고
    • EGR2 mutations in inherited neuropathies dominant-negatively inhibit myelin gene expression
    • Nagarajan R., Svaren J., Le N., Araki T., Watson M., Milbrandt J. EGR2 mutations in inherited neuropathies dominant-negatively inhibit myelin gene expression. Neuron 2001, 30:355-368.
    • (2001) Neuron , vol.30 , pp. 355-368
    • Nagarajan, R.1    Svaren, J.2    Le, N.3    Araki, T.4    Watson, M.5    Milbrandt, J.6
  • 115
    • 0032948117 scopus 로고    scopus 로고
    • Mutations in the peripheral myelin genes and associated genes in inherited peripheral neuropathies
    • Nelis E., Haites N., Van Broeckhoven C. Mutations in the peripheral myelin genes and associated genes in inherited peripheral neuropathies. Hum Mutat 1999, 13:11-28.
    • (1999) Hum Mutat , vol.13 , pp. 11-28
    • Nelis, E.1    Haites, N.2    Van Broeckhoven, C.3
  • 118
    • 0033485879 scopus 로고    scopus 로고
    • Altered molecular architecture of peripheral nerves in mice lacking the peripheral myelin protein 22 or connexin32
    • Neuberg D.H., Sancho S., Suter U. Altered molecular architecture of peripheral nerves in mice lacking the peripheral myelin protein 22 or connexin32. J Neurosci Res 1999, 58:612-623.
    • (1999) J Neurosci Res , vol.58 , pp. 612-623
    • Neuberg, D.H.1    Sancho, S.2    Suter, U.3
  • 119
    • 0035477457 scopus 로고    scopus 로고
    • Altered gene expression in Schwann cells of connexin32 knockout animals
    • Nicholson S.M., Gomes D., de Nechaud B., Bruzzone R. Altered gene expression in Schwann cells of connexin32 knockout animals. J Neurosci Res 2001, 66:23-36.
    • (2001) J Neurosci Res , vol.66 , pp. 23-36
    • Nicholson, S.M.1    Gomes, D.2    de Nechaud, B.3    Bruzzone, R.4
  • 120
    • 0034212644 scopus 로고    scopus 로고
    • Uncoupling of myelin assembly and schwann cell differentiation by transgenic overexpression of peripheral myelin protein 22
    • Niemann S., Sereda M.W., Suter U., Griffiths I.R., Nave K.A. Uncoupling of myelin assembly and schwann cell differentiation by transgenic overexpression of peripheral myelin protein 22. J Neurosci 2000, 20:4120-4128.
    • (2000) J Neurosci , vol.20 , pp. 4120-4128
    • Niemann, S.1    Sereda, M.W.2    Suter, U.3    Griffiths, I.R.4    Nave, K.A.5
  • 123
    • 0030991166 scopus 로고    scopus 로고
    • Upregulation of the endosomal-lysosomal pathway in the trembler-J neuropathy
    • Notterpek L., Shooter E.M., Snipes G.J. Upregulation of the endosomal-lysosomal pathway in the trembler-J neuropathy. J Neurosci 1997, 17:4190-4200.
    • (1997) J Neurosci , vol.17 , pp. 4190-4200
    • Notterpek, L.1    Shooter, E.M.2    Snipes, G.J.3
  • 124
    • 0036005810 scopus 로고    scopus 로고
    • Cell-intrinsic and cell-extrinsic cues regulating lineage decisions in multipotent neural crest-derived progenitor cells
    • Paratore P., Hagedorn L., Floris J., Hari L., Kleber M., Suter U., Sommer L. Cell-intrinsic and cell-extrinsic cues regulating lineage decisions in multipotent neural crest-derived progenitor cells. Int J Dev Biol 46: 193-200 (2002) 2002, 46:193-200.
    • (2002) Int J Dev Biol 46: 193-200 (2002) , vol.46 , pp. 193-200
    • Paratore, P.1    Hagedorn, L.2    Floris, J.3    Hari, L.4    Kleber, M.5    Suter, U.6    Sommer, L.7
  • 126
    • 0031037253 scopus 로고    scopus 로고
    • PMP-22 expression in the central nervous system of the embryonic mouse defines potential transverse segments and longitudinal columns
    • Paramantier E., Braun C., Thomas J.L., Peyron F., Martinez S., Zalc B. PMP-22 expression in the central nervous system of the embryonic mouse defines potential transverse segments and longitudinal columns. J Comp Neurol 1997, 378:159-172.
    • (1997) J Comp Neurol , vol.378 , pp. 159-172
    • Paramantier, E.1    Braun, C.2    Thomas, J.L.3    Peyron, F.4    Martinez, S.5    Zalc, B.6
  • 129
    • 0034004661 scopus 로고    scopus 로고
    • Protein zero gene expression is regulated by the glial transcription factor Sox10
    • Peirano R.I., Goerich D.E., Riethmacher D., Wegner M. Protein zero gene expression is regulated by the glial transcription factor Sox10. Mol Cell Biol 2000, 20:3198-3209.
    • (2000) Mol Cell Biol , vol.20 , pp. 3198-3209
    • Peirano, R.I.1    Goerich, D.E.2    Riethmacher, D.3    Wegner, M.4
  • 131
    • 0037115722 scopus 로고    scopus 로고
    • Effects of Charcot-Marie-Tooth-linked mutations of the neurofilament light subunit on intermediate filament formation
    • Perez-Olle R., Leung C.L., Liem R.K. Effects of Charcot-Marie-Tooth-linked mutations of the neurofilament light subunit on intermediate filament formation. J Cell Sci 2002, 115:4937-4946.
    • (2002) J Cell Sci , vol.115 , pp. 4937-4946
    • Perez-Olle, R.1    Leung, C.L.2    Liem, R.K.3
  • 133
    • 0032893517 scopus 로고    scopus 로고
    • Congenital hypomyelinating neuropathy: Two patients with long-term follow-up
    • Phillips J.P., Warner L.E., Lupski J.R., Garg B.P. Congenital hypomyelinating neuropathy: Two patients with long-term follow-up. Pediatr. Neurol. 1999, 20:226-232.
    • (1999) Pediatr. Neurol. , vol.20 , pp. 226-232
    • Phillips, J.P.1    Warner, L.E.2    Lupski, J.R.3    Garg, B.P.4
  • 137
    • 0037045408 scopus 로고    scopus 로고
    • Effects of mouse strain, position of integration and tetracycline analogue on the tetracycline conditional system in transgenic mice
    • Robertson A., Perea J., Tolmachova T., Thomas P.K., Huxley C. Effects of mouse strain, position of integration and tetracycline analogue on the tetracycline conditional system in transgenic mice. Gene 2002, 282:65-74.
    • (2002) Gene , vol.282 , pp. 65-74
    • Robertson, A.1    Perea, J.2    Tolmachova, T.3    Thomas, P.K.4    Huxley, C.5
  • 138
    • 0032758859 scopus 로고    scopus 로고
    • Development of early postnatal peripheral nerve abnormalities in Trembler-J and PMP22 transgenic mice
    • Robertson A.M., Huxley C., King R.H., Thomas P.K. Development of early postnatal peripheral nerve abnormalities in Trembler-J and PMP22 transgenic mice. J Anat 1999, 195:331-339.
    • (1999) J Anat , vol.195 , pp. 331-339
    • Robertson, A.M.1    Huxley, C.2    King, R.H.3    Thomas, P.K.4
  • 139
    • 0030996714 scopus 로고    scopus 로고
    • Abnormal Schwann cell/axon interactions in the Trembler-J mouse
    • Robertson A.M., King R.H., Muddle J.R., Thomas P.K. Abnormal Schwann cell/axon interactions in the Trembler-J mouse. J Anat 1997, 190:423-432.
    • (1997) J Anat , vol.190 , pp. 423-432
    • Robertson, A.M.1    King, R.H.2    Muddle, J.R.3    Thomas, P.K.4
  • 141
    • 0036070537 scopus 로고    scopus 로고
    • Aggresome formation in neuropathy models based on peripheral myelin protein 22 mutations
    • Ryan M.C., Shooter E.M., Notterpek L. Aggresome formation in neuropathy models based on peripheral myelin protein 22 mutations. Neurobiol Dis 2002, 10:109-118.
    • (2002) Neurobiol Dis , vol.10 , pp. 109-118
    • Ryan, M.C.1    Shooter, E.M.2    Notterpek, L.3
  • 142
    • 0033554346 scopus 로고    scopus 로고
    • Abnormal Schwann cell-axon interactions in CMT neuropathies. The effects of mutant Schwann cells on the axonal cytoskeleton and regenration-associated myelination
    • Sahenk Z. Abnormal Schwann cell-axon interactions in CMT neuropathies. The effects of mutant Schwann cells on the axonal cytoskeleton and regenration-associated myelination. Ann N Y Acad Sci 1999, 883:415-426.
    • (1999) Ann N Y Acad Sci , vol.883 , pp. 415-426
    • Sahenk, Z.1
  • 143
    • 0031783172 scopus 로고    scopus 로고
    • A novel PMP22 point mutation causing HNPP phenotype: Studies on nerve xenografts
    • Sahenk Z., Chen L., Freimer M. A novel PMP22 point mutation causing HNPP phenotype: Studies on nerve xenografts. Neurology 1998, 51:702-707.
    • (1998) Neurology , vol.51 , pp. 702-707
    • Sahenk, Z.1    Chen, L.2    Freimer, M.3
  • 144
    • 0032948050 scopus 로고    scopus 로고
    • Effects of PMP22 duplication and deletions on the axonal cytoskeleton
    • Sahenk Z., Chen L., Mendell J.R. Effects of PMP22 duplication and deletions on the axonal cytoskeleton. Ann Neurol 1999, 45:16-24.
    • (1999) Ann Neurol , vol.45 , pp. 16-24
    • Sahenk, Z.1    Chen, L.2    Mendell, J.R.3
  • 145
    • 0032889851 scopus 로고    scopus 로고
    • Identification of break points in mutated PMP22 gene in a new Trembler (Tr-Ncnp). mouse
    • Sakai Y., Nakabayashi O., Kikuchi T., Wada K. Identification of break points in mutated PMP22 gene in a new Trembler (Tr-Ncnp). mouse. Neuroscience 1999, 88:989-991.
    • (1999) Neuroscience , vol.88 , pp. 989-991
    • Sakai, Y.1    Nakabayashi, O.2    Kikuchi, T.3    Wada, K.4
  • 147
    • 0032815826 scopus 로고    scopus 로고
    • Distal axonopathy in peripheral nerves of PMP22-mutant mice
    • Sancho S., Magyar J.P., Aguzzi A., Suter U. Distal axonopathy in peripheral nerves of PMP22-mutant mice. Brain 1999, 122:1563-1577.
    • (1999) Brain , vol.122 , pp. 1563-1577
    • Sancho, S.1    Magyar, J.P.2    Aguzzi, A.3    Suter, U.4
  • 148
    • 0035173786 scopus 로고    scopus 로고
    • Regulation of Schwann cell proliferation and apoptosis in PMP22-deficient mice and mouse models of Charcot-Marie-Tooth disease type 1A
    • Sancho S., Young P., Suter U. Regulation of Schwann cell proliferation and apoptosis in PMP22-deficient mice and mouse models of Charcot-Marie-Tooth disease type 1A. Brain 2001, 124:2177-2187.
    • (2001) Brain , vol.124 , pp. 2177-2187
    • Sancho, S.1    Young, P.2    Suter, U.3
  • 149
    • 0032851256 scopus 로고    scopus 로고
    • High metabolism and subsequent elongation of 3-hydroxyeicosanoyl-CoA in very-long-chain fatty acid deficient PNS of Trembler mice
    • Sargueil F., Knoll A., Salles J., Garbay B., Cassagne C. High metabolism and subsequent elongation of 3-hydroxyeicosanoyl-CoA in very-long-chain fatty acid deficient PNS of Trembler mice. Neurosci Lett 1999, 273:29-32.
    • (1999) Neurosci Lett , vol.273 , pp. 29-32
    • Sargueil, F.1    Knoll, A.2    Salles, J.3    Garbay, B.4    Cassagne, C.5
  • 153
    • 0029615322 scopus 로고
    • Periaxin expression in myelinating Schwann cells: Modulation by axon-glial interactions and polarized localization during development
    • Scherer S.S., Xu Y.T., Bannerman P.G., Sherman D.L., Brophy P.J. Periaxin expression in myelinating Schwann cells: Modulation by axon-glial interactions and polarized localization during development. Development 1995, 121:4265-4273.
    • (1995) Development , vol.121 , pp. 4265-4273
    • Scherer, S.S.1    Xu, Y.T.2    Bannerman, P.G.3    Sherman, D.L.4    Brophy, P.J.5
  • 157
    • 0025601660 scopus 로고
    • Recombinant peripheral myelin protein Po confers both adhesion and neurite outgrowth-promoting properties
    • Schneider-Schaulies J., Brunn A.V., Schachner M. Recombinant peripheral myelin protein Po confers both adhesion and neurite outgrowth-promoting properties. J. Neuroscience Research 1990, 27:286-297.
    • (1990) J. Neuroscience Research , vol.27 , pp. 286-297
    • Schneider-Schaulies, J.1    Brunn, A.V.2    Schachner, M.3
  • 158
    • 0034114602 scopus 로고    scopus 로고
    • Charcot-Marie-Tooth neuropathy type 2 and P0 point mutations: Two novel amino acid substitutions (Asp61Gly; Tyr119Cys)., and a possible "hotspot" on Thr124Met
    • Senderek J., Hermanns B., Lehmann U., Bergmann C., Marx G., Kabus C., Timmerman V., Stoltenburg-Didinger G., Schroder J.M. Charcot-Marie-Tooth neuropathy type 2 and P0 point mutations: Two novel amino acid substitutions (Asp61Gly; Tyr119Cys)., and a possible "hotspot" on Thr124Met. Brain Pathol 2000, 10:235-248.
    • (2000) Brain Pathol , vol.10 , pp. 235-248
    • Senderek, J.1    Hermanns, B.2    Lehmann, U.3    Bergmann, C.4    Marx, G.5    Kabus, C.6    Timmerman, V.7    Stoltenburg-Didinger, G.8    Schroder, J.M.9
  • 161
    • 0030246987 scopus 로고    scopus 로고
    • Crystal structure of the extracellular domain from P0, the major structural protein of peripheral nerve myelin
    • Shapiro L., Doyle J.P., Hensley P., Colman D.R., Hendrickson W.A. Crystal structure of the extracellular domain from P0, the major structural protein of peripheral nerve myelin. Neuron 1996, 17:435-449.
    • (1996) Neuron , vol.17 , pp. 435-449
    • Shapiro, L.1    Doyle, J.P.2    Hensley, P.3    Colman, D.R.4    Hendrickson, W.A.5
  • 162
    • 0034968820 scopus 로고    scopus 로고
    • Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy
    • Sherman D.L., Fabrizi C., Gillespie C.S., Brophy P.J. Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy. Neuron 2001, 30:677-687.
    • (2001) Neuron , vol.30 , pp. 677-687
    • Sherman, D.L.1    Fabrizi, C.2    Gillespie, C.S.3    Brophy, P.J.4
  • 163
    • 0030611992 scopus 로고    scopus 로고
    • Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP)
    • Shy M.E., Arroyo E., Sladky J., Menichella D., Jiang H., Xu W., Kamholz J., Scherer S.S. Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP). J Neuropathol Exp Neurol 1997, 56:811-821.
    • (1997) J Neuropathol Exp Neurol , vol.56 , pp. 811-821
    • Shy, M.E.1    Arroyo, E.2    Sladky, J.3    Menichella, D.4    Jiang, H.5    Xu, W.6    Kamholz, J.7    Scherer, S.S.8
  • 165
    • 0037079142 scopus 로고    scopus 로고
    • The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease
    • Southwood C.M., Garbern J., Jiang W., Gow A. The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease. Neuron 2002, 36:585-596.
    • (2002) Neuron , vol.36 , pp. 585-596
    • Southwood, C.M.1    Garbern, J.2    Jiang, W.3    Gow, A.4
  • 167
    • 0030825570 scopus 로고    scopus 로고
    • An in-frame deletion in peripheral myelin protein-22 gene causes hypomyelination and cell death of the Schwann cells in the new Trembler mutant mice
    • Suh J.G., Ichihara N., Saigoh K., Nakabayashi O., Yamanishi T., Tanaka K., Wada K., Kikuchi T. An in-frame deletion in peripheral myelin protein-22 gene causes hypomyelination and cell death of the Schwann cells in the new Trembler mutant mice. Neuroscience 1997, 79:735-744.
    • (1997) Neuroscience , vol.79 , pp. 735-744
    • Suh, J.G.1    Ichihara, N.2    Saigoh, K.3    Nakabayashi, O.4    Yamanishi, T.5    Tanaka, K.6    Wada, K.7    Kikuchi, T.8
  • 170
    • 0033554318 scopus 로고    scopus 로고
    • Transgenic mouse models of CMT1A and HNPP
    • Suter U., Nave K.A. Transgenic mouse models of CMT1A and HNPP. Ann N Y Acad Sci 1999, 883:247-253.
    • (1999) Ann N Y Acad Sci , vol.883 , pp. 247-253
    • Suter, U.1    Nave, K.A.2
  • 173
    • 0027459799 scopus 로고
    • Progress in the molecular understanding of hereditary peripheral neuropathies reveals new insights into the biology of the peripheral nervous system
    • Suter U., Welcher A.A., Snipes G.J. Progress in the molecular understanding of hereditary peripheral neuropathies reveals new insights into the biology of the peripheral nervous system. Trends Neurosci 1993, 16:50-56.
    • (1993) Trends Neurosci , vol.16 , pp. 50-56
    • Suter, U.1    Welcher, A.A.2    Snipes, G.J.3
  • 175
    • 0027484361 scopus 로고
    • Perinatal lethality and defects in hindbrain development in mice homozygous for a targeted mutation of the zinc finger gene Krox20
    • Swiatek P.J., Gridley T. Perinatal lethality and defects in hindbrain development in mice homozygous for a targeted mutation of the zinc finger gene Krox20. Genes Dev 1993, 7:2071-2084.
    • (1993) Genes Dev , vol.7 , pp. 2071-2084
    • Swiatek, P.J.1    Gridley, T.2
  • 176
    • 0036193126 scopus 로고    scopus 로고
    • Conditional, floxed allele of the Krox20 gene
    • Taillebourg E., Buart S., Charnay P. Conditional, floxed allele of the Krox20 gene. Genesis 2002, 32:112-113.
    • (2002) Genesis , vol.32 , pp. 112-113
    • Taillebourg, E.1    Buart, S.2    Charnay, P.3
  • 178
    • 0033778125 scopus 로고    scopus 로고
    • Membrane topology of peripheral myelin protein 22
    • Taylor V., Zgraggen C., Naef R., Suter U. Membrane topology of peripheral myelin protein 22. J Neurosci Res 2000, 62:15-27.
    • (2000) J Neurosci Res , vol.62 , pp. 15-27
    • Taylor, V.1    Zgraggen, C.2    Naef, R.3    Suter, U.4
  • 179
    • 0031240077 scopus 로고    scopus 로고
    • High incidence of spontaneous and chemically induced liver tumors in mice deficient for connexin32
    • Temme A., Buchmann A., Gabriel H.D., Nelles E., Schwarz M., Willecke K. High incidence of spontaneous and chemically induced liver tumors in mice deficient for connexin32. Curr Biol 1997, 7:713-716.
    • (1997) Curr Biol , vol.7 , pp. 713-716
    • Temme, A.1    Buchmann, A.2    Gabriel, H.D.3    Nelles, E.4    Schwarz, M.5    Willecke, K.6
  • 180
    • 0037039362 scopus 로고    scopus 로고
    • Differential aggregation of the Trembler and Trembler J mutants of peripheral myelin protein 22
    • Tobler A.R., Liu N., Mueller L., Shooter E.M. Differential aggregation of the Trembler and Trembler J mutants of peripheral myelin protein 22. Proc Natl Acad Sci U S A 2002, 99:483-488.
    • (2002) Proc Natl Acad Sci U S A , vol.99 , pp. 483-488
    • Tobler, A.R.1    Liu, N.2    Mueller, L.3    Shooter, E.M.4
  • 181
    • 0033559844 scopus 로고    scopus 로고
    • Transport of Trembler-J mutant peripheral myelin protein 22 is blocked in the intermediate compartment and affects the transport of the wild-type protein by direct interaction
    • Tobler A.R., Notterpek L., Naef R., Taylor V., Suter U., Shooter E.M. Transport of Trembler-J mutant peripheral myelin protein 22 is blocked in the intermediate compartment and affects the transport of the wild-type protein by direct interaction. J Neurosci 1999, 19:2027-2036.
    • (1999) J Neurosci , vol.19 , pp. 2027-2036
    • Tobler, A.R.1    Notterpek, L.2    Naef, R.3    Taylor, V.4    Suter, U.5    Shooter, E.M.6
  • 183
    • 0030724815 scopus 로고    scopus 로고
    • Hereditary neuromyotonia: A mouse model associated with deficiency or increased gene dosage of the PMP22 gene
    • Toyka K.V., Zielasek J., Ricker K., Adlkofer K., Suter U. Hereditary neuromyotonia: A mouse model associated with deficiency or increased gene dosage of the PMP22 gene. J Neurol Neurosurg Psychiatry 1997, 63:812-813.
    • (1997) J Neurol Neurosurg Psychiatry , vol.63 , pp. 812-813
    • Toyka, K.V.1    Zielasek, J.2    Ricker, K.3    Adlkofer, K.4    Suter, U.5
  • 184
    • 0033754159 scopus 로고    scopus 로고
    • A novel mutation of desert hedgehog in a patient with 46,XY partial gonadal dysgenesis accompanied by minifascicular neuropathy
    • Umehara F., Tate G., Itoh K., Yamaguchi N., Douchi T., Mitsuya T., Osame M. A novel mutation of desert hedgehog in a patient with 46,XY partial gonadal dysgenesis accompanied by minifascicular neuropathy. Am J Hum Genet 2000, 67:1302-1305.
    • (2000) Am J Hum Genet , vol.67 , pp. 1302-1305
    • Umehara, F.1    Tate, G.2    Itoh, K.3    Yamaguchi, N.4    Douchi, T.5    Mitsuya, T.6    Osame, M.7
  • 188
    • 0034019478 scopus 로고    scopus 로고
    • Expression pattern of a Krox-20/Cre knock-in allele in the developing hindbrain, bones, and peripheral nervous system
    • Voiculescu O., Charnay P., Schneider-Maunoury S. Expression pattern of a Krox-20/Cre knock-in allele in the developing hindbrain, bones, and peripheral nervous system. Genesis 2000, 26:123-126.
    • (2000) Genesis , vol.26 , pp. 123-126
    • Voiculescu, O.1    Charnay, P.2    Schneider-Maunoury, S.3
  • 189
    • 0029608908 scopus 로고
    • Hypomyelination alters K+ channel expression in mouse mutants shiverer and Trembler
    • Wang H., Allen M.L., Grigg J.J., Noebels J.L., Tempel B.L. Hypomyelination alters K+ channel expression in mouse mutants shiverer and Trembler. Neuron 1995, 15:1337-1347.
    • (1995) Neuron , vol.15 , pp. 1337-1347
    • Wang, H.1    Allen, M.L.2    Grigg, J.J.3    Noebels, J.L.4    Tempel, B.L.5
  • 191
    • 0031943222 scopus 로고    scopus 로고
    • Mutations in the early growth response 2 (EGR2) gene are associated with hereditary myelinopathies
    • Warner L.E., Mancias P., Butler I.J., McDonald C.M., Keppen L., Koob K.G., Lupski J.R. Mutations in the early growth response 2 (EGR2) gene are associated with hereditary myelinopathies. Nat Genet 1998, 18:382-384.
    • (1998) Nat Genet , vol.18 , pp. 382-384
    • Warner, L.E.1    Mancias, P.2    Butler, I.J.3    McDonald, C.M.4    Keppen, L.5    Koob, K.G.6    Lupski, J.R.7
  • 192
    • 0032797721 scopus 로고    scopus 로고
    • Functional consequences of mutations in the early growth response 2 gene (EGR2). correlate with severity of human myelinopathies
    • Warner L.E., Svaren J., Milbrandt J., Lupski J.R. Functional consequences of mutations in the early growth response 2 gene (EGR2). correlate with severity of human myelinopathies. Hum Mol Genet 1999, 8:1245-1251.
    • (1999) Hum Mol Genet , vol.8 , pp. 1245-1251
    • Warner, L.E.1    Svaren, J.2    Milbrandt, J.3    Lupski, J.R.4
  • 193
    • 0035680926 scopus 로고    scopus 로고
    • Multiple splice isoforms of proteolipid M6B in neurons and oligodendrocytes
    • Werner H., Dimou L., Klugmann M., Pfeiffer S., Nave K.A. Multiple splice isoforms of proteolipid M6B in neurons and oligodendrocytes. Mol Cell Neurosci 2001, 18:593-605.
    • (2001) Mol Cell Neurosci , vol.18 , pp. 593-605
    • Werner, H.1    Dimou, L.2    Klugmann, M.3    Pfeiffer, S.4    Nave, K.A.5
  • 194
    • 0033554363 scopus 로고    scopus 로고
    • Characterization of targeted connexin32-deficient mice: A model for the human Charcot-Marie-Tooth (X-type). inherited disease
    • Willecke K., Temme A., Teubner B., Ott T. Characterization of targeted connexin32-deficient mice: A model for the human Charcot-Marie-Tooth (X-type). inherited disease. Ann N Y Acad Sci 1999, 883:302-309.
    • (1999) Ann N Y Acad Sci , vol.883 , pp. 302-309
    • Willecke, K.1    Temme, A.2    Teubner, B.3    Ott, T.4
  • 195
    • 0037072748 scopus 로고    scopus 로고
    • Epithelial membrane proteins induce membrane blebbing and interact with the P2X7 receptor C-terminus
    • press
    • Wilson H.L., Wilson S.A., Surprenant A., North R.A. Epithelial membrane proteins induce membrane blebbing and interact with the P2X7 receptor C-terminus. J Biol Chem 2002, in press.
    • (2002) J Biol Chem
    • Wilson, H.L.1    Wilson, S.A.2    Surprenant, A.3    North, R.A.4
  • 196
    • 0001215716 scopus 로고
    • Inherited recurrent neuropathy
    • WB Saunders, New York, P.J. Dyck, P.K. Thomas, J.W. Griffin, P.A. Low, J.F. Poduslo (Eds.)
    • Windebank A.J. Inherited recurrent neuropathy. Peripheral Neuropathy 1993, 1094-1136. WB Saunders, New York. P.J. Dyck, P.K. Thomas, J.W. Griffin, P.A. Low, J.F. Poduslo (Eds.).
    • (1993) Peripheral Neuropathy , pp. 1094-1136
    • Windebank, A.J.1
  • 197
    • 0029797249 scopus 로고    scopus 로고
    • Dominant-negative effect on adhesion by myelin Po protein truncated in its cytoplasmic domain
    • Wong M.H., Filbin M.T. Dominant-negative effect on adhesion by myelin Po protein truncated in its cytoplasmic domain. J. Cell Biol. 1996, 134:1531-1541.
    • (1996) J. Cell Biol. , vol.134 , pp. 1531-1541
    • Wong, M.H.1    Filbin, M.T.2
  • 199
    • 0034973979 scopus 로고    scopus 로고
    • Do Schwann cells stop, DR(o)P2, and roll?
    • Wrabetz L., Feltri M.L. Do Schwann cells stop, DR(o)P2, and roll?. Neuron 2001, 30:642-644.
    • (2001) Neuron , vol.30 , pp. 642-644
    • Wrabetz, L.1    Feltri, M.L.2
  • 202
    • 0032521342 scopus 로고    scopus 로고
    • Myelin-associated glycoprotein is a myelin signal that modulates the caliber of myelinated axons
    • Yin X., Crawford T.O., Griffin J.W., Tu P., Lee V.M., Li C., Roder J., Trapp B.D. Myelin-associated glycoprotein is a myelin signal that modulates the caliber of myelinated axons. J Neurosci 1998, 18:1953-1962.
    • (1998) J Neurosci , vol.18 , pp. 1953-1962
    • Yin, X.1    Crawford, T.O.2    Griffin, J.W.3    Tu, P.4    Lee, V.M.5    Li, C.6    Roder, J.7    Trapp, B.D.8
  • 203
    • 0034611007 scopus 로고    scopus 로고
    • Schwann cell myelination requires timely and precise targeting of P(0). protein
    • Yin X., Kidd G.J., Wrabetz L., Feltri M.L., Messing A., Trapp B.D. Schwann cell myelination requires timely and precise targeting of P(0). protein. J Cell Biol 2000, 148:1009-1020.
    • (2000) J Cell Biol , vol.148 , pp. 1009-1020
    • Yin, X.1    Kidd, G.J.2    Wrabetz, L.3    Feltri, M.L.4    Messing, A.5    Trapp, B.D.6
  • 204
    • 0036900365 scopus 로고    scopus 로고
    • Identification of novel sequence variants in the neurofilament-light gene in a Japanese population: Analysis of Charcot-Marie-Tooth disease patients and normal individuals
    • Yoshihara T., Yamamoto M., Hattori N., Misu K., Mori K., Koike H., Sobue G. Identification of novel sequence variants in the neurofilament-light gene in a Japanese population: Analysis of Charcot-Marie-Tooth disease patients and normal individuals. J Peripher Nerv Syst 2002, 7:221-224.
    • (2002) J Peripher Nerv Syst , vol.7 , pp. 221-224
    • Yoshihara, T.1    Yamamoto, M.2    Hattori, N.3    Misu, K.4    Mori, K.5    Koike, H.6    Sobue, G.7
  • 205
    • 0032125516 scopus 로고    scopus 로고
    • Myelin Po protein mutated at Cys21 has a dominant-negative effect on adhesion of wild type Po
    • Zhang K., Filbin M.T. Myelin Po protein mutated at Cys21 has a dominant-negative effect on adhesion of wild type Po. J. Neurosci Res. 1998, 53:1-6.
    • (1998) J. Neurosci Res. , vol.53 , pp. 1-6
    • Zhang, K.1    Filbin, M.T.2
  • 207
    • 0031263931 scopus 로고    scopus 로고
    • Delayed maturation of regenerating myelinated axons in mice lacking neurofilaments
    • Zhu Q., Couillard-Despres S., Julien J.P. Delayed maturation of regenerating myelinated axons in mice lacking neurofilaments. Exp Neurol 1997, 148:299-316.
    • (1997) Exp Neurol , vol.148 , pp. 299-316
    • Zhu, Q.1    Couillard-Despres, S.2    Julien, J.P.3
  • 208
    • 0034020892 scopus 로고    scopus 로고
    • Neuromyotonia in mice with hereditary myelinopathies
    • Zielasek J., Martini R., Suter U., Toyka K.V. Neuromyotonia in mice with hereditary myelinopathies. Muscle Nerve 2000, 23:696-701.
    • (2000) Muscle Nerve , vol.23 , pp. 696-701
    • Zielasek, J.1    Martini, R.2    Suter, U.3    Toyka, K.V.4
  • 209
    • 0029666023 scopus 로고    scopus 로고
    • Functional Abnormalities in P-0-Deficient Mice Resemble Human Hereditary Neuropathies Linked to P-0 Gene Mutations
    • Zielasek J., Martini R., Toyka K.V. Functional Abnormalities in P-0-Deficient Mice Resemble Human Hereditary Neuropathies Linked to P-0 Gene Mutations. Muscle & Nerve 1996, 19:946-952.
    • (1996) Muscle & Nerve , vol.19 , pp. 946-952
    • Zielasek, J.1    Martini, R.2    Toyka, K.V.3


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