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Volumn 1001, Issue 1-2, 2004, Pages 37-50

Late onset Tay-Sachs disease in mice with targeted disruption of the Hexa gene: Behavioral changes and pathology of the central nervous system

Author keywords

Behavioral phenotype of Tay Sachs mouse model; GM2 gangliosidosis; Hexa gene mutation; Lysosomal storage disease; Mouse model of Tay Sachs disease; Neuropathology of Tay Sachs mouse model

Indexed keywords

GANGLIOSIDE GM2;

EID: 10744226948     PISSN: 00068993     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.brainres.2003.11.067     Document Type: Article
Times cited : (26)

References (37)
  • 1
    • 0033214561 scopus 로고    scopus 로고
    • Catabolism of asialo-GM2 in man and mouse. Specificity of human/mouse chimeric GM2 activator proteins
    • Bertoni C., Li Y.T., Li S.C. Catabolism of asialo-GM2 in man and mouse. Specificity of human/mouse chimeric GM2 activator proteins. J. Biol. Chem. 274:1999;28612-28618.
    • (1999) J. Biol. Chem. , vol.274 , pp. 28612-28618
    • Bertoni, C.1    Li, Y.T.2    Li, S.C.3
  • 2
    • 0014073241 scopus 로고
    • Motor function and the corticospinal tracts in the dog and raccoon
    • Buxton D.F., Goodman D.C. Motor function and the corticospinal tracts in the dog and raccoon. J. Comp. Neurol. 129:1967;341-360.
    • (1967) J. Comp. Neurol. , vol.129 , pp. 341-360
    • Buxton, D.F.1    Goodman, D.C.2
  • 3
    • 0033013928 scopus 로고    scopus 로고
    • Gait analysis in the mouse
    • Clarke K.A., Still J. Gait analysis in the mouse. Physiol. Behav. 66:1999;723-729.
    • (1999) Physiol. Behav. , vol.66 , pp. 723-729
    • Clarke, K.A.1    Still, J.2
  • 4
    • 1242333579 scopus 로고    scopus 로고
    • Behaviour, neurology and electrophysiology
    • G.J. Krinke. New York: Academic Press
    • Classen W. Behaviour, neurology and electrophysiology. Krinke G.J. The Laboratory Rat. 2000;419-436 Academic Press, New York.
    • (2000) The Laboratory Rat , pp. 419-436
    • Classen, W.1
  • 7
    • 0002055041 scopus 로고    scopus 로고
    • Tremors: Differential diagnosis, neurophysiology, and pharmacology
    • J. Jankovic, & E. Tolosa. Baltimore: Williams and Wilkins
    • Deuschl G., Krack P. Tremors: differential diagnosis, neurophysiology, and pharmacology. Jankovic J., Tolosa E. Parkinson's Disease and Movement Disorders. 1998;419-452 Williams and Wilkins, Baltimore.
    • (1998) Parkinson's Disease and Movement Disorders , pp. 419-452
    • Deuschl, G.1    Krack, P.2
  • 11
    • 0030782030 scopus 로고    scopus 로고
    • Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases
    • Huang J.Q., Trasler J.M., Igdoura S., Michaud J., Hanal N., Gravel R.A. Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases. Hum. Mol. Genet. 6:1997;1879-1885.
    • (1997) Hum. Mol. Genet. , vol.6 , pp. 1879-1885
    • Huang, J.Q.1    Trasler, J.M.2    Igdoura, S.3    Michaud, J.4    Hanal, N.5    Gravel, R.A.6
  • 12
    • 0014424692 scopus 로고
    • Comprehensive observational assessment: Ia. A systematic, quantitative procedure for assessing the behavioral and physiologic state of the mouse
    • Irwin S. Comprehensive observational assessment: Ia. A systematic, quantitative procedure for assessing the behavioral and physiologic state of the mouse. Psychopharmacologia (Berl.). 13:1966;222-257.
    • (1966) Psychopharmacologia (Berl.) , vol.13 , pp. 222-257
    • Irwin, S.1
  • 14
    • 0035168688 scopus 로고    scopus 로고
    • Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation
    • Jeyakumar M., Norflus F., Tifft C.J., Cortina-Borja M., Butters T.D., Proia R.L., Perry V.H., Dwek R.A., Platt F.M. Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation. Blood. 97:2001;327-329.
    • (2001) Blood , vol.97 , pp. 327-329
    • Jeyakumar, M.1    Norflus, F.2    Tifft, C.J.3    Cortina-Borja, M.4    Butters, T.D.5    Proia, R.L.6    Perry, V.H.7    Dwek, R.A.8    Platt, F.M.9
  • 16
    • 0014273386 scopus 로고
    • The quantiative measurement of motor inco-ordination in naive mice using an acelerating rotarod
    • Jones B.J., Roberts D.J. The quantiative measurement of motor inco-ordination in naive mice using an acelerating rotarod. J. Pharm. Pharmacol. 20:1968;302-304.
    • (1968) J. Pharm. Pharmacol. , vol.20 , pp. 302-304
    • Jones, B.J.1    Roberts, D.J.2
  • 17
    • 0035110728 scopus 로고    scopus 로고
    • Substrate reduction therapy for glycosphingolipid storage disorders
    • Lachmann R.H., Platt F.M. Substrate reduction therapy for glycosphingolipid storage disorders. Expert Opin. Investig. Drugs. 10:2001;455-466.
    • (2001) Expert Opin. Investig. Drugs , vol.10 , pp. 455-466
    • Lachmann, R.H.1    Platt, F.M.2
  • 18
    • 0035890683 scopus 로고    scopus 로고
    • Degradation of G(M1) and G(M2) by mammalian sialidases
    • Li S.C., Li Y.T., Moriya S., Miyagi T. Degradation of G(M1) and G(M2) by mammalian sialidases. Biochem. J. 360:2001;233-237.
    • (2001) Biochem. J. , vol.360 , pp. 233-237
    • Li, S.C.1    Li, Y.T.2    Moriya, S.3    Miyagi, T.4
  • 22
    • 0042447491 scopus 로고    scopus 로고
    • The cerebellum: A model for construction of a cortex
    • Mason C., Sotelo C. The cerebellum: a model for construction of a cortex. Perspect. Dev. Neurobiol. 5:1997;1-95.
    • (1997) Perspect. Dev. Neurobiol. , vol.5 , pp. 1-95
    • Mason, C.1    Sotelo, C.2
  • 23
    • 0027173341 scopus 로고
    • Utility of a neurobehavioral screening battery for differentiating the effects of two pyrethroids, permethrin and cypermethrin
    • McDaniel K.L., Moser V.C. Utility of a neurobehavioral screening battery for differentiating the effects of two pyrethroids, permethrin and cypermethrin. Neurotoxicol. Teratol. 15:1993;71-83.
    • (1993) Neurotoxicol. Teratol. , vol.15 , pp. 71-83
    • McDaniel, K.L.1    Moser, V.C.2
  • 24
    • 0029052252 scopus 로고
    • A multidisciplinary approach to toxicological screening: III. Neurobehavioral toxicity
    • Moser V.C., Cheek B.M., MacPhail R.C. A multidisciplinary approach to toxicological screening: III. Neurobehavioral toxicity. J. Toxicol. Environ. Health. 45:1995;173-210.
    • (1995) J. Toxicol. Environ. Health , vol.45 , pp. 173-210
    • Moser, V.C.1    Cheek, B.M.2    MacPhail, R.C.3
  • 27
    • 0033980069 scopus 로고    scopus 로고
    • Distribution of enzyme-bearing cells in GM2 gangliosidosis mice: Regionally specific pattern of cellular infiltration following bone marrow transplantation
    • Oya Y., Proia R.L., Norflus F., Tifft C.J., Langaman C., Suzuki K. Distribution of enzyme-bearing cells in GM2 gangliosidosis mice: regionally specific pattern of cellular infiltration following bone marrow transplantation. Acta Neuropathol. (Berl). 99:2000;161-168.
    • (2000) Acta Neuropathol. (Berl) , vol.99 , pp. 161-168
    • Oya, Y.1    Proia, R.L.2    Norflus, F.3    Tifft, C.J.4    Langaman, C.5    Suzuki, K.6
  • 31
    • 0017588481 scopus 로고
    • Specificity of human liver hexosaminidases a and B against glycosphingolipids GM2 and GA2. Purification of the enzymes by affinity chromatography employing specific elution, Hoppe Seylers
    • Sandhoff K., Conzelmann E., Nehrkorn H. Specificity of human liver hexosaminidases A and B against glycosphingolipids GM2 and GA2. Purification of the enzymes by affinity chromatography employing specific elution, Hoppe Seylers. Z. Physiol. Chem. 358:1977;779-787.
    • (1977) Z. Physiol. Chem. , vol.358 , pp. 779-787
    • Sandhoff, K.1    Conzelmann, E.2    Nehrkorn, H.3
  • 35
    • 0015425113 scopus 로고
    • Studies on the substrate specificity of hexosaminidase a and B from liver
    • Wenger D.A., Okada S., O'Brien J.S. Studies on the substrate specificity of hexosaminidase A and B from liver. Arch. Biochem. Biophys. 153:1972;116-129.
    • (1972) Arch. Biochem. Biophys. , vol.153 , pp. 116-129
    • Wenger, D.A.1    Okada, S.2    O'Brien, J.S.3
  • 37
    • 0031594717 scopus 로고    scopus 로고
    • Specificity of mouse GM2 activator protein and beta-N- acetylhexosaminidases a and B. Similarities and differences with their human counterparts in the catabolism of GM2
    • Yuziuk J.A., Bertoni C., Beccari T., Orlacchio A., Wu Y.Y., Li S.C., Li Y.T. Specificity of mouse GM2 activator protein and beta-N- acetylhexosaminidases A and B. Similarities and differences with their human counterparts in the catabolism of GM2. J. Biol. Chem. 273:1998;66-72.
    • (1998) J. Biol. Chem. , vol.273 , pp. 66-72
    • Yuziuk, J.A.1    Bertoni, C.2    Beccari, T.3    Orlacchio, A.4    Wu, Y.Y.5    Li, S.C.6    Li, Y.T.7


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.