메뉴 건너뛰기




Volumn 12, Issue 1, 2000, Pages 27-32

Transient neuromotor phenotype in transgenic spastic mice expressing low levels of glycine receptor β-subunit: An animal model of startle disease

Author keywords

Glycine receptor; Hereditary hyperekplexia; spa spa TG456 mice; Startle syndrome

Indexed keywords

GLYCINE RECEPTOR; MESSENGER RNA; RECEPTOR SUBTYPE;

EID: 0033967649     PISSN: 0953816X     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1460-9568.2000.00877.x     Document Type: Article
Times cited : (24)

References (43)
  • 1
    • 0019225609 scopus 로고
    • Startle disease or hyperekplexia; further delineation of the syndrome
    • Andermann, F., Keene, D.L., Andermann, E., Qeesney, L. & F. (1980) Startle disease or hyperekplexia; further delineation of the syndrome. Brain, 103, 985-997.
    • (1980) Brain , vol.103 , pp. 985-997
    • Andermann, F.1    Keene, D.L.2    Andermann, E.3    Qeesney, L.F.4
  • 2
    • 0022531593 scopus 로고
    • The glycine receptor deficiency of the mutant mouse spastic: Evidence for normal glycine receptor structure and localization
    • Becker, C.-M., Hermans-Borgmeyer, I., Schmitt, B. & Betz, H. (1986) The glycine receptor deficiency of the mutant mouse spastic: evidence for normal glycine receptor structure and localization. J. Neurosci., 5, 1358-1364.
    • (1986) J. Neurosci. , vol.5 , pp. 1358-1364
    • Becker, C.-M.1    Hermans-Borgmeyer, I.2    Schmitt, B.3    Betz, H.4
  • 3
    • 0024225307 scopus 로고
    • Glycine receptor heterogeneity in rat spinal cord during postnatal development
    • Becker, C.-M., Hoch, W. & Betz, H. (1988) Glycine receptor heterogeneity in rat spinal cord during postnatal development. EMBO J., 7, 3717-3726.
    • (1988) EMBO J. , vol.7 , pp. 3717-3726
    • Becker, C.-M.1    Hoch, W.2    Betz, H.3
  • 4
    • 0026522823 scopus 로고
    • Isoform-selective deficit of glycine receptors in the mouse mutant spastic
    • Becker, C.-M., Schmieden, V., Tarroni, P., Strasser, U. & Betz, H. (1992) Isoform-selective deficit of glycine receptors in the mouse mutant spastic. Neuron, 8, 283-289.
    • (1992) Neuron , vol.8 , pp. 283-289
    • Becker, C.-M.1    Schmieden, V.2    Tarroni, P.3    Strasser, U.4    Betz, H.5
  • 5
    • 0027409976 scopus 로고
    • Expression of inhibitory glycine receptors in postnatal rat cerebral cortex
    • Becker, C.-M., Betz, H. & Schröder, H. (1993) Expression of inhibitory glycine receptors in postnatal rat cerebral cortex. Brain Res., 26, 220-226.
    • (1993) Brain Res. , vol.26 , pp. 220-226
    • Becker, C.-M.1    Betz, H.2    Schröder, H.3
  • 6
    • 0027097784 scopus 로고
    • Structure and function of inhibitory glycine receptors
    • Betz, H. (1992) Structure and function of inhibitory glycine receptors. Q. Rev. Biophys., 25, 283-289.
    • (1992) Q. Rev. Biophys. , vol.25 , pp. 283-289
    • Betz, H.1
  • 8
    • 0024583109 scopus 로고
    • Startle disease or hyperekplexia: Adolescent onset and response to valproate
    • Dooley, J.M. & Andermann, F. (1989) Startle disease or hyperekplexia: adolescent onset and response to valproate. Pediatr. Neurol., 2, 126-127.
    • (1989) Pediatr. Neurol. , vol.2 , pp. 126-127
    • Dooley, J.M.1    Andermann, F.2
  • 9
    • 0025063668 scopus 로고
    • Transgenic mice expressing β-galactosidase in mature neurons under neuron-specific enolase prornotor control
    • Forss-Petter, S., Danielson, P.E., Catsicas, S., Battenberg, E., Price, J., Nerenberg, M. & Sutcliffe, J.G. (1990) Transgenic mice expressing β-galactosidase in mature neurons under neuron-specific enolase prornotor control. Neuron, 5, 187-197.
    • (1990) Neuron , vol.5 , pp. 187-197
    • Forss-Petter, S.1    Danielson, P.E.2    Catsicas, S.3    Battenberg, E.4    Price, J.5    Nerenberg, M.6    Sutcliffe, J.G.7
  • 10
    • 0020435972 scopus 로고
    • Recombinant genomes which express chloramphenicol acetyltransferase in mammalian cells
    • Gorman, C.M., Moffat, L.F. & Horward, B.H. (1982) Recombinant genomes which express chloramphenicol acetyltransferase in mammalian cells. Mol. Cell. Biol., 2, 1044-1051.
    • (1982) Mol. Cell. Biol. , vol.2 , pp. 1044-1051
    • Gorman, C.M.1    Moffat, L.F.2    Horward, B.H.3
  • 11
    • 0025095280 scopus 로고
    • Alpha subunit variants of the human glycine receptor: Primary structures, functional expression and chromosomal localization of the corresponding genes
    • Grenningloh, G., Schmieden, V., Schofield, P.R., Seeburg, P.H., Siddique, T., Mohandas, T.K., Becker, C.-M. & Betz, H. (1990a) Alpha subunit variants of the human glycine receptor: primary structures, functional expression and chromosomal localization of the corresponding genes. EMBO J., 9, 771-776.
    • (1990) EMBO J. , vol.9 , pp. 771-776
    • Grenningloh, G.1    Schmieden, V.2    Schofield, P.R.3    Seeburg, P.H.4    Siddique, T.5    Mohandas, T.K.6    Becker, C.-M.7    Betz, H.8
  • 14
    • 0029923342 scopus 로고    scopus 로고
    • Low level expression of glycine receptor β subunit transgene is sufficient for phenotype correction in spastic mice
    • Hartenstein, B., Schenkel, J., Kuhse, J., Besenbeck, B., Kling, C., Becker, C.-M., Betz, H. & Weiher, H. (1996) Low level expression of glycine receptor β subunit transgene is sufficient for phenotype correction in spastic mice. EMBO J., 15, 1275-1282.
    • (1996) EMBO J. , vol.15 , pp. 1275-1282
    • Hartenstein, B.1    Schenkel, J.2    Kuhse, J.3    Besenbeck, B.4    Kling, C.5    Becker, C.-M.6    Betz, H.7    Weiher, H.8
  • 15
    • 0024721995 scopus 로고
    • Primary cultures of mouse spinal cord express the neonatal isoform of the inhibitory glycine receptor
    • Hoch, W., Betz, H. & Becker, C.-M. (1989) Primary cultures of mouse spinal cord express the neonatal isoform of the inhibitory glycine receptor. Neuron, 3, 339-348.
    • (1989) Neuron , vol.3 , pp. 339-348
    • Hoch, W.1    Betz, H.2    Becker, C.-M.3
  • 18
    • 0028175530 scopus 로고
    • Glycine receptor β-subunit gene mutation in spastic mouse associated with LINE-1 element insertion
    • Kingsmore, S.F., Giros, B., Suh, D., Bieniarz, M., Caron, M.G. & Seidin, M.F. (1994) Glycine receptor β-subunit gene mutation in spastic mouse associated with LINE-1 element insertion. Nature Genet., 7, 136-141.
    • (1994) Nature Genet. , vol.7 , pp. 136-141
    • Kingsmore, S.F.1    Giros, B.2    Suh, D.3    Bieniarz, M.4    Caron, M.G.5    Seidin, M.F.6
  • 19
    • 0028848501 scopus 로고
    • Targeting of glycine receptor subunits to gephyrin-rich domains in transfected human embryonic kidney cells
    • Kirsch, J., Kuhse, J. & Betz, H. (1995) Targeting of glycine receptor subunits to gephyrin-rich domains in transfected human embryonic kidney cells. Mol Cell. Neurosci., 6, 450-461.
    • (1995) Mol Cell. Neurosci. , vol.6 , pp. 450-461
    • Kirsch, J.1    Kuhse, J.2    Betz, H.3
  • 20
    • 0025649599 scopus 로고
    • Identification and functional expression of a novel ligand binding subunit of the inhibitory glycine receptor
    • Kuhse, J., Schmieden, V. & Betz, H. (1990) Identification and functional expression of a novel ligand binding subunit of the inhibitory glycine receptor. J. Biol. Chem., 265, 22317-22320.
    • (1990) J. Biol. Chem. , vol.265 , pp. 22317-22320
    • Kuhse, J.1    Schmieden, V.2    Betz, H.3
  • 21
    • 0027716526 scopus 로고
    • Assembly of the inhibitory glycine receptor: Identification of amino acid sequence motifs governing subunit stoichiometry
    • Kuhse, J., Laube, B., Magalei, D. & Betz, H. (1993) Assembly of the inhibitory glycine receptor: identification of amino acid sequence motifs governing subunit stoichiometry. Neuron, 11, 1049-1056.
    • (1993) Neuron , vol.11 , pp. 1049-1056
    • Kuhse, J.1    Laube, B.2    Magalei, D.3    Betz, H.4
  • 22
    • 0020693750 scopus 로고
    • Hyperekplexia
    • Kurczynski, T.W. (1983) Hyperekplexia. Arch. Neurol., 40, 246-248.
    • (1983) Arch. Neurol. , vol.40 , pp. 246-248
    • Kurczynski, T.W.1
  • 23
    • 0001202076 scopus 로고
    • Conserved quaternary structure of ligand gated ion channels: The postsynaptic glycine receptor is a pentamer
    • Langosch, D., Thomas, L. & Betz, H. (1988) Conserved quaternary structure of ligand gated ion channels: the postsynaptic glycine receptor is a pentamer. Proc. Natl Acad. Sci. USA, 85, 7394-7398.
    • (1988) Proc. Natl Acad. Sci. USA , vol.85 , pp. 7394-7398
    • Langosch, D.1    Thomas, L.2    Betz, H.3
  • 24
    • 0028016521 scopus 로고
    • Decreased agonist affinity and chloride conductance of mutant glycine receptors associated with human hereditary hyperekplexia
    • Langosch, D., Laube, B., Rundström, N., Schmieden, V., Bormann, J. & Betz, H. (1994) Decreased agonist affinity and chloride conductance of mutant glycine receptors associated with human hereditary hyperekplexia. EMBO J., 13, 4223-4228.
    • (1994) EMBO J. , vol.13 , pp. 4223-4228
    • Langosch, D.1    Laube, B.2    Rundström, N.3    Schmieden, V.4    Bormann, J.5    Betz, H.6
  • 26
    • 0030849296 scopus 로고    scopus 로고
    • Assessment of locomotor activity, acoustic and tactile startle in inbred mouse strains and Fl hybrids: Implications of genetic background for single gene and quantitative trait loci analyses
    • Logue, S.F., Owen, E.H., Rasmussen, D.L. & Wehner, J.M. (1997) Assessment of locomotor activity, acoustic and tactile startle in inbred mouse strains and Fl hybrids: implications of genetic background for single gene and quantitative trait loci analyses. Neuroscience, 80, 1075-1086.
    • (1997) Neuroscience , vol.80 , pp. 1075-1086
    • Logue, S.F.1    Owen, E.H.2    Rasmussen, D.L.3    Wehner, J.M.4
  • 27
    • 0025734108 scopus 로고
    • Widespread expression of glycine receptor subunit mRNAs in the adult and developing rat brain
    • Malosio, M.-L., Marqèze-Pouey, B., Kuhse, J. & Betz, H. (1991) Widespread expression of glycine receptor subunit mRNAs in the adult and developing rat brain. EMBO J., 10, 2401-2409.
    • (1991) EMBO J. , vol.10 , pp. 2401-2409
    • Malosio, M.-L.1    Marqèze-Pouey, B.2    Kuhse, J.3    Betz, H.4
  • 29
    • 0029126893 scopus 로고
    • Studies of porcine and human sperm suggesting a role for a sperm glycine receptor/Cl channel in the zona pelluzida-initiated acrosome reaction
    • Melendrez, C.S. & Meizel, S. (1995) Studies of porcine and human sperm suggesting a role for a sperm glycine receptor/Cl channel in the zona pelluzida-initiated acrosome reaction. Biol. Reprod., 53, 676-683.
    • (1995) Biol. Reprod. , vol.53 , pp. 676-683
    • Melendrez, C.S.1    Meizel, S.2
  • 30
    • 0030601089 scopus 로고    scopus 로고
    • Immunochemical identification of the glycine receptor/Cl-channel in porcine sperm
    • Melendrez, C.S. & Meizel, S. (1996) Immunochemical identification of the glycine receptor/Cl-channel in porcine sperm. Biochem. Biaphys. Res. Commun., 223, 675-678.
    • (1996) Biochem. Biaphys. Res. Commun. , vol.223 , pp. 675-678
    • Melendrez, C.S.1    Meizel, S.2
  • 31
    • 0029124881 scopus 로고
    • Identification of a gephyrin binding motif on the glycine receptor β subunit
    • Meyer, G., Kirsch, J., Betz, H. & Langosch, D. (1995) Identification of a gephyrin binding motif on the glycine receptor β subunit. Neuron, 15, 563-572.
    • (1995) Neuron , vol.15 , pp. 563-572
    • Meyer, G.1    Kirsch, J.2    Betz, H.3    Langosch, D.4
  • 32
    • 0027996651 scopus 로고
    • The spastic mouse: Aberrant splicing of glycine receptor β subunit mRNA caused by intronic insertion of L1 element
    • Mülhardt, C., Fischer, M., Gass, P., Simon-Chazottes, D., Guenet, J.-L., Kuhse, J., Betz, H. & Becker, C.-M. (1994) The spastic mouse: aberrant splicing of glycine receptor β subunit mRNA caused by intronic insertion of LI element. Neuron, 13, 1003-1015.
    • (1994) Neuron , vol.13 , pp. 1003-1015
    • Mülhardt, C.1    Fischer, M.2    Gass, P.3    Simon-Chazottes, D.4    Guenet, J.-L.5    Kuhse, J.6    Betz, H.7    Becker, C.-M.8
  • 33
    • 0019779878 scopus 로고
    • Solubilization of the glycine receptor from rat spinal cord
    • Pfeiffer, F. & Betz, H. (1981) Solubilization of the glycine receptor from rat spinal cord. Brain Res., 226, 273-279.
    • (1981) Brain Res. , vol.226 , pp. 273-279
    • Pfeiffer, F.1    Betz, H.2
  • 34
    • 0021751227 scopus 로고
    • Monoclonal antibodies and peptide mapping reveal structural similarities between the subunits of the glycine receptor of rat spinal cord
    • Pfeiffer, F., Simler, R., Grenningloh, G. & Betz, H. (1984) Monoclonal antibodies and peptide mapping reveal structural similarities between the subunits of the glycine receptor of rat spinal cord. Proc. Natl Acad Sci. USA, 81, 7224-7227.
    • (1984) Proc. Natl Acad Sci. USA , vol.81 , pp. 7224-7227
    • Pfeiffer, F.1    Simler, R.2    Grenningloh, G.3    Betz, H.4
  • 35
    • 0028361803 scopus 로고
    • Startle disease mutations reduce the agonist sensitivity of the human inhibitory glycine receptor
    • Rajendra, S., Lynch, J.W., Pierce, K.D., French, C.R., Barry, H.B. & Schofield, P.R. (1994) Startle disease mutations reduce the agonist sensitivity of the human inhibitory glycine receptor. J. Biol. Chem., 269, 18739-18742.
    • (1994) J. Biol. Chem. , vol.269 , pp. 18739-18742
    • Rajendra, S.1    Lynch, J.W.2    Pierce, K.D.3    French, C.R.4    Barry, H.B.5    Schofield, P.R.6
  • 37
    • 0026651547 scopus 로고
    • Startle disease, or hyperekplexia: Response to clonazepam and assignment of the gene (STHE) to chromosome 5q by linkage analysis
    • Ryan, S.G., Sherman, S.L., Terry, J.C., Sparkes, R.S., Torres, M.C. & Mackey, R.W. (1992) Startle disease, or hyperekplexia: response to clonazepam and assignment of the gene (STHE) to chromosome 5q by linkage analysis. Ann. Neurol., 31, 663-668.
    • (1992) Ann. Neurol. , vol.31 , pp. 663-668
    • Ryan, S.G.1    Sherman, S.L.2    Terry, J.C.3    Sparkes, R.S.4    Torres, M.C.5    Mackey, R.W.6
  • 40
    • 0026607904 scopus 로고
    • Agonist pharmacology of neonatal and adult glycine receptor alpha subunits: Identification of amino acid residues involved in taurine activation
    • Schmieden, V., Kuhse, J. & Betz, H. (1992) Agonist pharmacology of neonatal and adult glycine receptor alpha subunits: identification of amino acid residues involved in taurine activation. EMBO J., 11, 2025-2032.
    • (1992) EMBO J. , vol.11 , pp. 2025-2032
    • Schmieden, V.1    Kuhse, J.2    Betz, H.3
  • 42
    • 0000069083 scopus 로고
    • Hyperekplexia: A hereditary startle syndrome
    • Suhren, O., Bruyn, G.W. & Tuynman, J.A. (1966) Hyperekplexia: a hereditary startle syndrome. J. Neurol. Sci., 3, 577-605.
    • (1966) J. Neurol. Sci. , vol.3 , pp. 577-605
    • Suhren, O.1    Bruyn, G.W.2    Tuynman, J.A.3
  • 43
    • 0020052195 scopus 로고
    • Glycine receptor alteration in the mutant mouse spastic
    • White, W.F. & Heller, A.H. (1982) Glycine receptor alteration in the mutant mouse spastic. Nature, 298, 655-657.
    • (1982) Nature , vol.298 , pp. 655-657
    • White, W.F.1    Heller, A.H.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.