-
1
-
-
0014806327
-
Globoid cell leucodystrophy (Krabbe's disease): Deficiency of galactocerebroside beta-galactosidase
-
Suzuki, K and Suzuki, Y (1970). Globoid cell leucodystrophy (Krabbe's disease): Deficiency of galactocerebroside beta-galactosidase. Proc Natl Acad Sci USA 66: 302-309.
-
(1970)
Proc Natl Acad Sci USA
, vol.66
, pp. 302-309
-
-
Suzuki, K.1
Suzuki, Y.2
-
2
-
-
0030964590
-
Molecular genetics of Krabbe disease (globoid cell leukodystrophy): Diagnostic and clinical implications
-
Wenger, DA, Rafi, MA and Luzi, P (1997). Molecular genetics of Krabbe disease (globoid cell leukodystrophy): Diagnostic and clinical implications. Hum Mutat 10: 268-279.
-
(1997)
Hum Mutat
, vol.10
, pp. 268-279
-
-
Wenger, D.A.1
Rafi, M.A.2
Luzi, P.3
-
3
-
-
0019274883
-
The Twitcher mouse: An enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease)
-
Kobayashi, T, Yamanaka, T, Jacobs, JM, Teixeira, F and Suzuki, K (1980). The Twitcher mouse: An enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease). Brain Res 202: 479-483.
-
(1980)
Brain Res
, vol.202
, pp. 479-483
-
-
Kobayashi, T.1
Yamanaka, T.2
Jacobs, J.M.3
Teixeira, F.4
Suzuki, K.5
-
4
-
-
0028785109
-
Murine model of genetic demyelinating disease: The twitcher mouse
-
Suzuki, K and Taniike, M (1995). Murine model of genetic demyelinating disease: The twitcher mouse. Microsc Res Tech 32: 204-214.
-
(1995)
Microsc Res Tech
, vol.32
, pp. 204-214
-
-
Suzuki, K.1
Taniike, M.2
-
5
-
-
0034799137
-
Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements
-
Shen, JS, Watabe, K, Ohashi, T and Eto, Y (2001). Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements. Gene Ther 8: 1081-1087.
-
(2001)
Gene Ther
, vol.8
, pp. 1081-1087
-
-
Shen, J.S.1
Watabe, K.2
Ohashi, T.3
Eto, Y.4
-
6
-
-
17644374493
-
AAV-mediated expression of galactocerebrosidase in brain results in attenuated symptoms and extended life span in murine models of globoid cell leukodystrophy
-
Rafi, MA et al. (2005). AAV-mediated expression of galactocerebrosidase in brain results in attenuated symptoms and extended life span in murine models of globoid cell leukodystrophy. Mol Ther 11: 734-744.
-
(2005)
Mol Ther
, vol.11
, pp. 734-744
-
-
Rafi, M.A.1
-
7
-
-
23844451244
-
AAV2/5 vector expressing galactocerebrosidase ameliorates CNS disease in the murine model of globoid-cell leukodystrophy more efficiently than AAV2
-
Lin, D et al. (2005). AAV2/5 vector expressing galactocerebrosidase ameliorates CNS disease in the murine model of globoid-cell leukodystrophy more efficiently than AAV2. Mol Ther 12: 422-430.
-
(2005)
Mol Ther
, vol.12
, pp. 422-430
-
-
Lin, D.1
-
8
-
-
0021249707
-
Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse
-
Yeager, AM, Brennan, S, Tiffany, C, Moser, HW and Santos, GW (1984). Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse. Science 225: 1052-1054.
-
(1984)
Science
, vol.225
, pp. 1052-1054
-
-
Yeager, A.M.1
Brennan, S.2
Tiffany, C.3
Moser, H.W.4
Santos, G.W.5
-
9
-
-
0023928896
-
Effect of bone marrow transplantation on enzyme levels and clinical course in the neurologically affected twitcher mouse
-
Hoogerbrugge, PM, Poorthuis, BJ, Romme, AE, van de Kamp, JJ, Wagemaker, G and van Bekkum, DW (1988). Effect of bone marrow transplantation on enzyme levels and clinical course in the neurologically affected twitcher mouse. J Clin Invest 81: 1790-1794.
-
(1988)
J Clin Invest
, vol.81
, pp. 1790-1794
-
-
Hoogerbrugge, P.M.1
Poorthuis, B.J.2
Romme, A.E.3
van de Kamp, J.J.4
Wagemaker, G.5
van Bekkum, D.W.6
-
10
-
-
0023900289
-
The twitcher mouse. Central nervous system pathology after bone marrow transplantation
-
Suzuki, K, Hoogerbrugge, PM, Poorthuis, BJ, Bekkum, DW and Suzuki, K (1988). The twitcher mouse. Central nervous system pathology after bone marrow transplantation. Lab Invest 58: 302-309.
-
(1988)
Lab Invest
, vol.58
, pp. 302-309
-
-
Suzuki, K.1
Hoogerbrugge, P.M.2
Poorthuis, B.J.3
Bekkum, D.W.4
Suzuki, K.5
-
11
-
-
0032537062
-
Hematopoietic stem-cell transplantation in globoid-cell leukodystrophy
-
Krivit, W et al. (1998). Hematopoietic stem-cell transplantation in globoid-cell leukodystrophy. N Engl J Med 338: 1119-1126.
-
(1998)
N Engl J Med
, vol.338
, pp. 1119-1126
-
-
Krivit, W.1
-
12
-
-
20844453744
-
Transplantation of umbilical-cord blood in babies with infantile Krabbe's disease
-
Escolar, ML et al. (2005). Transplantation of umbilical-cord blood in babies with infantile Krabbe's disease. N Engl J Med 352: 2069-2081.
-
(2005)
N Engl J Med
, vol.352
, pp. 2069-2081
-
-
Escolar, M.L.1
-
13
-
-
3242781657
-
Analysis of galactocerebrosidase activity in the mouse brain by a new histological staining method
-
Dolcetta, D et al. (2004). Analysis of galactocerebrosidase activity in the mouse brain by a new histological staining method. J Neurosci Res 77: 462-464.
-
(2004)
J Neurosci Res
, vol.77
, pp. 462-464
-
-
Dolcetta, D.1
-
14
-
-
0027258694
-
Treatment of murine mucopolysaccharidosis type VII by syngeneic bone marrow transplantation in neonates
-
Sands, MS et al. (1993). Treatment of murine mucopolysaccharidosis type VII by syngeneic bone marrow transplantation in neonates. Lab Invest 68: 676-686.
-
(1993)
Lab Invest
, vol.68
, pp. 676-686
-
-
Sands, M.S.1
-
15
-
-
0023176251
-
Neurological and neurobehavioral development of the mutant "twitcher" mouse
-
Olmstead, CE (1987). Neurological and neurobehavioral development of the mutant "twitcher" mouse. Behav Brain Res 25: 143-153.
-
(1987)
Behav Brain Res
, vol.25
, pp. 143-153
-
-
Olmstead, C.E.1
-
16
-
-
0022035244
-
Genetic galactosylceramidase deficiency (globoid cell leukodystrophy, Krabbe disease) in different mammalian species
-
Suzuki, K and Suzuki, K (1985). Genetic galactosylceramidase deficiency (globoid cell leukodystrophy, Krabbe disease) in different mammalian species. Neurochem Pathol 3: 53-68.
-
(1985)
Neurochem Pathol
, vol.3
, pp. 53-68
-
-
Suzuki, K.1
Suzuki, K.2
-
17
-
-
0030898926
-
Murine mucopolysaccharidosis type VII: Long term therapeutic effects of enzyme replacement and enzyme replacement followed by bone marrow transplantation
-
Sands, MS et al. (1997). Murine mucopolysaccharidosis type VII: Long term therapeutic effects of enzyme replacement and enzyme replacement followed by bone marrow transplantation. J Clin Invest 99: 1596-1605.
-
(1997)
J Clin Invest
, vol.99
, pp. 1596-1605
-
-
Sands, M.S.1
-
18
-
-
0035168688
-
Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation
-
Jeyakumar, M et al. (2001). Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation. Blood 97: 327-329.
-
(2001)
Blood
, vol.97
, pp. 327-329
-
-
Jeyakumar, M.1
-
19
-
-
0034718598
-
Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation
-
Wada, R, Tifft, CJ and Proia, RL (2000). Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation. Proc Natl Acad Sci USA 97: 10954-10959.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 10954-10959
-
-
Wada, R.1
Tifft, C.J.2
Proia, R.L.3
-
20
-
-
0037452606
-
Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB
-
Ohmi, K, Greenberg, DS, Rajavel, KS, Ryazantsev, S, Li, HH and Neufeld, EF (2003). Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB. Proc Natl Acad Sci USA 100: 1902-1907.
-
(2003)
Proc Natl Acad Sci USA
, vol.100
, pp. 1902-1907
-
-
Ohmi, K.1
Greenberg, D.S.2
Rajavel, K.S.3
Ryazantsev, S.4
Li, H.H.5
Neufeld, E.F.6
-
21
-
-
15244355223
-
Specific induction of macrophage inflammatory protein 1-alpha in glial cells of Sandhoff disease model mice associated with accumulation of N-acetylhexosaminyl glycoconjugates
-
Tsuji, D et al. (2005). Specific induction of macrophage inflammatory protein 1-alpha in glial cells of Sandhoff disease model mice associated with accumulation of N-acetylhexosaminyl glycoconjugates. J Neurochem 92: 1497-1507.
-
(2005)
J Neurochem
, vol.92
, pp. 1497-1507
-
-
Tsuji, D.1
-
22
-
-
0026536975
-
The twitcher mouse: Immunocytochemical study of Ia expression in macrophages
-
Higashi, Y, Komiyama, A and Suzuki, K (1992). The twitcher mouse: immunocytochemical study of Ia expression in macrophages. J Neuropathol Exp Neurol 51: 47-57.
-
(1992)
J Neuropathol Exp Neurol
, vol.51
, pp. 47-57
-
-
Higashi, Y.1
Komiyama, A.2
Suzuki, K.3
-
23
-
-
0027471683
-
Proliferation of microglia/macrophages in the demyelinating CNS and PNS of twitcher mouse
-
Ohno, M, Komiyama, A, Martin, PM and Suzuki, K (1993). Proliferation of microglia/macrophages in the demyelinating CNS and PNS of twitcher mouse. Brain Res 602: 268-274.
-
(1993)
Brain Res
, vol.602
, pp. 268-274
-
-
Ohno, M.1
Komiyama, A.2
Martin, P.M.3
Suzuki, K.4
-
24
-
-
0027994601
-
Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy
-
Matsushima, GK et al. (1994). Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy. Cell 78: 645-656.
-
(1994)
Cell
, vol.78
, pp. 645-656
-
-
Matsushima, G.K.1
-
25
-
-
0034759421
-
Expression of immune-related molecules is downregulated in twitcher mice following bone marrow transplantation
-
Wu, YP, McMahon, EJ, Matsuda, J, Suzuki, K, Matsushima, GK and Suzuki, K (2001). Expression of immune-related molecules is downregulated in twitcher mice following bone marrow transplantation. J Neuropathol Exp Neurol 60: 1062-1074.
-
(2001)
J Neuropathol Exp Neurol
, vol.60
, pp. 1062-1074
-
-
Wu, Y.P.1
McMahon, E.J.2
Matsuda, J.3
Suzuki, K.4
Matsushima, G.K.5
Suzuki, K.6
-
26
-
-
16844362608
-
Mechanisms of suppression by suppressor T cells
-
von Boehmer, H (2005). Mechanisms of suppression by suppressor T cells. Nat Immunol 6: 338-344.
-
(2005)
Nat Immunol
, vol.6
, pp. 338-344
-
-
von Boehmer, H.1
-
27
-
-
0023212302
-
Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): Effects on levels of galactosylceramidase, psychosine, and galactocerebrosides
-
Ichioka, T, Kishimoto, Y, Brennan, S, Santos, GW and Yeager, AM (1987). Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): Effects on levels of galactosylceramidase, psychosine, and galactocerebrosides. Proc Natl Acad Sci USA 84: 4259-4263.
-
(1987)
Proc Natl Acad Sci USA
, vol.84
, pp. 4259-4263
-
-
Ichioka, T.1
Kishimoto, Y.2
Brennan, S.3
Santos, G.W.4
Yeager, A.M.5
-
28
-
-
0023735493
-
Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation
-
Kondo, A, Hoogerbrugge, PM, Suzuki, K, Poorthuis, BJ, Van Bekkum, DW and Suzuki, K (1988). Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation. Brain Res 460: 178-183.
-
(1988)
Brain Res
, vol.460
, pp. 178-183
-
-
Kondo, A.1
Hoogerbrugge, P.M.2
Suzuki, K.3
Poorthuis, B.J.4
Van Bekkum, D.W.5
Suzuki, K.6
-
29
-
-
0019473590
-
Peripheral nerve grafts in hereditary leukodystrophic mutant mice (twitcher)
-
Scaravilli, F and Jacobs, JM (1981). Peripheral nerve grafts in hereditary leukodystrophic mutant mice (twitcher). Nature 290: 56-58.
-
(1981)
Nature
, vol.290
, pp. 56-58
-
-
Scaravilli, F.1
Jacobs, J.M.2
-
30
-
-
0023947989
-
DNA amplification of adeno-associated virus as a response to cellular genotoxic stress
-
Yalkinoglu, AO, Heilbronn, R, Burkle, A, Schlehofer, JR and zur Hausen, H (1988). DNA amplification of adeno-associated virus as a response to cellular genotoxic stress. Cancer Res 48: 3123-3129.
-
(1988)
Cancer Res
, vol.48
, pp. 3123-3129
-
-
Yalkinoglu, A.O.1
Heilbronn, R.2
Burkle, A.3
Schlehofer, J.R.4
zur Hausen, H.5
-
31
-
-
0027997836
-
DNA-damaging agents greatly increase the transduction of nondividing cells by adeno-associated virus vectors
-
Alexander, IE, Russell, DW and Miller, AD (1994). DNA-damaging agents greatly increase the transduction of nondividing cells by adeno-associated virus vectors. J Virol 68: 8282-8287.
-
(1994)
J Virol
, vol.68
, pp. 8282-8287
-
-
Alexander, I.E.1
Russell, D.W.2
Miller, A.D.3
-
32
-
-
13344286310
-
Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease
-
Sakai, N et al. (1996). Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease. J Neurochem 66: 1118-1124.
-
(1996)
J Neurochem
, vol.66
, pp. 1118-1124
-
-
Sakai, N.1
-
33
-
-
0033020597
-
Percutaneous intravenous injection in neonatal mice
-
Sands, MS and Barker, JE (1999). Percutaneous intravenous injection in neonatal mice. Lab Anim Sci 49: 328-330.
-
(1999)
Lab Anim Sci
, vol.49
, pp. 328-330
-
-
Sands, M.S.1
Barker, J.E.2
-
34
-
-
0001590529
-
Screening for lysosomal disorders
-
In: Hommes FA (ed). Wiley-Liss: New York
-
Wenger, D (1991). Screening for lysosomal disorders In: Hommes FA (ed). Techniques in Diagnostics Human Biochemical Genetics. Wiley-Liss: New York, pp 587-617.
-
(1991)
Techniques in Diagnostics Human Biochemical Genetics
, pp. 587-617
-
-
Wenger, D.1
-
35
-
-
0031255352
-
Behavioral and functional analysis of mouse phenotype: SHIRPA a proposed protocol for comprehensive phenotype assessment
-
Rogers, DC, Fisher, EM, Brown, SD, Peters, J, Hunter, AJ and Martin, JE (1997). Behavioral and functional analysis of mouse phenotype: SHIRPA a proposed protocol for comprehensive phenotype assessment. Mamm Genome 8: 711-713.
-
(1997)
Mamm Genome
, vol.8
, pp. 711-713
-
-
Rogers, D.C.1
Fisher, E.M.2
Brown, S.D.3
Peters, J.4
Hunter, A.J.5
Martin, J.E.6
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