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Volumn 79, Issue 1, 2003, Pages 1-8

Evolving perspective of the pathogenesis of globoid cell leukodystrophy (Krabbe disease)

Author keywords

Galactosylceramide; Globoid cell leukodystrophy; Krabbe disease; Pathogenesis; Psychosine hypothesis; Saposin A

Indexed keywords

CANIS FAMILIARIS;

EID: 0037245175     PISSN: 03862208     EISSN: None     Source Type: Journal    
DOI: 10.2183/pjab.79B.1     Document Type: Review
Times cited : (17)

References (40)
  • 1
    • 0001775305 scopus 로고
    • A new familial, infantile form of diffuse brain sclerosis
    • Krabbe, K. (1916) A new familial, infantile form of diffuse brain sclerosis. Brain 39, 74-114.
    • (1916) Brain , vol.39 , pp. 74-114
    • Krabbe, K.1
  • 2
    • 0012664877 scopus 로고    scopus 로고
    • Lysosomal disease
    • (eds. Graham, D. I., and Lantos, P. L). Edward Arnold, London
    • Suzuki, K., and Suzuki, K. (2002) Lysosomal disease. In Greenfield's Neuropathology (eds. Graham, D. I., and Lantos, P. L). Edward Arnold, London, pp 653-735.
    • (2002) Greenfield's Neuropathology , pp. 653-735
    • Suzuki, K.1    Suzuki, K.2
  • 3
    • 0001276892 scopus 로고
    • Studies in globoid (Krabbe) leukodystrophy III. Significance of experimentally-produced globoid-like elements in rat white matter and spleen
    • Austin, J. H., and Lehfeldt, D. (1965) Studies in globoid (Krabbe) leukodystrophy III. Significance of experimentally-produced globoid-like elements in rat white matter and spleen. J. Neuropathol. Exp. Neurol. 24, 265-289.
    • (1965) J. Neuropathol. Exp. Neurol. , vol.24 , pp. 265-289
    • Austin, J.H.1    Lehfeldt, D.2
  • 4
    • 0014899868 scopus 로고
    • Ultrastructure of experimentally produced globoid cells in the rat
    • Andrews, J. M., and Menkes, J. H. (1970) Ultrastructure of experimentally produced globoid cells in the rat. Exp. Neurol 29, 483-493
    • (1970) Exp. Neurol. , vol.29 , pp. 483-493
    • Andrews, J.M.1    Menkes, J.H.2
  • 5
    • 0014900558 scopus 로고
    • Ultrastructural study of experimental globoid cells
    • Suzuki, K. (1970) Ultrastructural study of experimental globoid cells. Lab. Invest. 23, 612-619.
    • (1970) Lab. Invest. , vol.23 , pp. 612-619
    • Suzuki, K.1
  • 6
    • 0014806327 scopus 로고
    • Globoid cell leucodystrophy (Krabbe's disease): Deficiency of galactocerebroside β-galactosidase
    • Suzuki, K., and Suzuki, Y. (1970) Globoid cell leucodystrophy (Krabbe's disease): Deficiency of galactocerebroside β-galactosidase. Proc. Natl. Acad. Sci, U. S A 66, 302-309.
    • (1970) Proc. Natl. Acad. Sci, U. S. A. , vol.66 , pp. 302-309
    • Suzuki, K.1    Suzuki, Y.2
  • 7
    • 0014857292 scopus 로고
    • Studies in globoid leukodystrophy: Enzymatic and lipid findings in the canine form
    • Suzuki, Y., Austin, J., Armstrong, D., Suzuki, K., Schlenker, J., and Fletcher, T. (1970) Studies in globoid leukodystrophy: Enzymatic and lipid findings in the canine form. Exp Neurol. 29, 65-75.
    • (1970) Exp. Neurol. , vol.29 , pp. 65-75
    • Suzuki, Y.1    Austin, J.2    Armstrong, D.3    Suzuki, K.4    Schlenker, J.5    Fletcher, T.6
  • 8
    • 0014845669 scopus 로고
    • Globoid cell leukodystrophy (Krabbe's disease): Isolation of myelin with normal glycolipid composition
    • Eto, Y., Suzuki, K., and Suzuki, K. (1970) Globoid cell leukodystrophy (Krabbe's disease): Isolation of myelin with normal glycolipid composition. J. Lipid Res. 11, 473-479.
    • (1970) J. Lipid Res. , vol.11 , pp. 473-479
    • Eto, Y.1    Suzuki, K.2    Suzuki, K.3
  • 9
    • 0012723879 scopus 로고
    • Zwei Typen von Cerebrosid-Schwefelsaureestern als sog. "Pralipoide" und Speichersubstanzen bei der Leukodystrophie, Typ Scholz (metachromatische Form der diffusen Sklerose) Hpper-Seylers
    • Jatzkewitz, H. (1958) Zwei Typen von Cerebrosid-Schwefelsaureestern als sog. "Pralipoide" und Speichersubstanzen bei der Leukodystrophie, Typ Scholz (metachromatische Form der diffusen Sklerose) Hpper-Seylers Z. Physiol Chem. 311, 279-282.
    • (1958) Z. Physiol. Chem. , vol.311 , pp. 279-282
    • Jatzkewitz, H.1
  • 10
    • 0013850515 scopus 로고
    • Myelin membrane: A molecular abnormality
    • O'Brien, J. S., and Sampson, E. L (1965) Myelin membrane: A molecular abnormality. Science 150, 1613-1614.
    • (1965) Science , vol.150 , pp. 1613-1614
    • O'Brien, J.S.1    Sampson, E.L.2
  • 11
    • 0012758879 scopus 로고
    • The variation in the chemical composition in myelin in disease and during development
    • Norton, W T. (1967) The variation in the chemical composition in myelin in disease and during development. Charing Cross Hosp. Gazette 8, 3-8.
    • (1967) Charing Cross Hosp. Gazette , vol.8 , pp. 3-8
    • Norton, W.T.1
  • 12
    • 0001767013 scopus 로고
    • Inborn lysosomal disease
    • Hers, H G (1966) Inborn lysosomal disease. Gastroenterology 48, 625-633.
    • (1966) Gastroenterology , vol.48 , pp. 625-633
    • Hers, H.G.1
  • 13
    • 0015496948 scopus 로고
    • Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase
    • Miyatake, T., and Suzuki, K. (1972) Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase. Biochem. Biophys. Res. Commun. 48, 538-543.
    • (1972) Biochem. Biophys. Res. Commun. , vol.48 , pp. 538-543
    • Miyatake, T.1    Suzuki, K.2
  • 14
    • 0031932297 scopus 로고    scopus 로고
    • Twenty five years of the psychosine hypothesis: A personal perspective of its history and present status
    • Suzuki, K. (1998) Twenty five years of the psychosine hypothesis: A personal perspective of its history and present status. Neurochem. Res. 23, 251-259.
    • (1998) Neurochem. Res. , vol.23 , pp. 251-259
    • Suzuki, K.1
  • 15
    • 0035873272 scopus 로고    scopus 로고
    • A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene causes a late-onset, slowly progressive form of globoid cell leukodystrophy in the mouse
    • Matsuda, J., Vanier, M. T., Saito, Y., Tohyama, J., Suzuki. K., and Suzuki, K. (2001) A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene causes a late-onset, slowly progressive form of globoid cell leukodystrophy in the mouse Hum. Mol Genet. 10, 1191-1199.
    • (2001) Hum. Mol. Genet. , vol.10 , pp. 1191-1199
    • Matsuda, J.1    Vanier, M.T.2    Saito, Y.3    Tohyama, J.4    Suzuki, K.5    Suzuki, K.6
  • 17
    • 0014931194 scopus 로고
    • Tubules of globoid cell leukodystrophy. A right-handed helix
    • Yunis, E. J., and Lee, R. E. (1970) Tubules of globoid cell leukodystrophy. A right-handed helix. Science 169, 64-66.
    • (1970) Science , vol.169 , pp. 64-66
    • Yunis, E.J.1    Lee, R.E.2
  • 18
    • 0016832398 scopus 로고
    • Chemical pathology of Krabbe's disease. III. Ceramide hexosides and gangliosides of brain
    • Vanier, M.-T., and Svennerhohn, L (1975) Chemical pathology of Krabbe's disease. III. Ceramide hexosides and gangliosides of brain. Acta Paediat. Scand. 64, 641-648
    • (1975) Acta Paediat. Scand. , vol.64 , pp. 641-648
    • Vanier, M.-T.1    Svennerhohn, L.2
  • 19
    • 0018821962 scopus 로고
    • Krabbe disease: A galactosylsphingosine (psychosine) lipidosis
    • Svennerholm, L., Vanier, M.-T., and Månsson, J -E (1980) Krabbe disease: A galactosylsphingosine (psychosine) lipidosis. J. Lipid Res. 21, 53-64
    • (1980) J. Lipid Res. , vol.21 , pp. 53-64
    • Svennerholm, L.1    Vanier, M.-T.2    Månsson, J.E.3
  • 20
    • 0016915389 scopus 로고
    • Chemical pathology of Krabbe disease: The occurrence of psychosine and other neutral sphingoglycolipids
    • (eds Volk, B. W., and Schneck, L.). Plenum Press, New York
    • Vanier, M -T, and Svennerholm, L. (1976) Chemical pathology of Krabbe disease: The occurrence of psychosine and other neutral sphingoglycolipids In Current Trends in Sphingolipidoses and Allied Disorders (eds Volk, B. W., and Schneck, L.). Plenum Press, New York, pp. 115-126.
    • (1976) Current Trends in Sphingolipidoses and Allied Disorders , pp. 115-126
    • Vanier, M.T.1    Svennerholm, L.2
  • 21
    • 0016906418 scopus 로고
    • Studies on the pathogenesis of Krabbe's leukodystrophy: Cellular reaction of the brain to exogenous galactosylsphingosine, monogalactosyl diglyceride and lactosylceramide
    • (eds. Volk, B. W., and Schneck, L.). Plenum Press, New York
    • Suzuki, K., Tanaka, H, and Suzuki, K. (1976) Studies on the pathogenesis of Krabbe's leukodystrophy: Cellular reaction of the brain to exogenous galactosylsphingosine, monogalactosyl diglyceride and lactosylceramide. In Current Trends in Sphingolipidoses and Allied Disorders (eds. Volk, B. W., and Schneck, L.). Plenum Press, New York, pp. 99-113.
    • (1976) Current Trends in Sphingolipidoses and Allied Disorders , pp. 99-113
    • Suzuki, K.1    Tanaka, H.2    Suzuki, K.3
  • 22
    • 0000708535 scopus 로고
    • Physiological activity of psychosine
    • Taketomi, T., and Nishimura, K. (1964) Physiological activity of psychosine. Jap. J. Exp. Med. 34, 255-265.
    • (1964) Jap. J. Exp. Med. , vol.34 , pp. 255-265
    • Taketomi, T.1    Nishimura, K.2
  • 23
    • 0021339782 scopus 로고
    • Progressive accumulation of toxic metabolite in a genetic leukodystrophy
    • Igisu, H., and Suzuki, K. (1984) Progressive accumulation of toxic metabolite in a genetic leukodystrophy. Science 224, 753-755.
    • (1984) Science , vol.224 , pp. 753-755
    • Igisu, H.1    Suzuki, K.2
  • 24
    • 0023138332 scopus 로고
    • Lysosphingolipids inhibit protein kinase C: Implications for the sphingolipidoses
    • Hannun, Y. A, and Bell, R. M. (1987) Lysosphingolipids inhibit protein kinase C: Implications for the sphingolipidoses. Science 235, 670-674.
    • (1987) Science , vol.235 , pp. 670-674
    • Hannun, Y.A.1    Bell, R.M.2
  • 25
    • 0019274883 scopus 로고
    • The twitcher mouse. An enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease)
    • Kobayashi, T., Yamanaka, T., Jacobs, J. M., 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
  • 26
    • 0035510055 scopus 로고    scopus 로고
    • Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: Estrogen appears to be a critical factor
    • Matsuda, J., Vanier, M. T., Saito, Y., Suzuki, K., and Suzuki, K. (2001) Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: estrogen appears to be a critical factor Hum. Mol Genet. 10, 2709-2715.
    • (2001) Hum. Mol. Genet. , vol.10 , pp. 2709-2715
    • Matsuda, J.1    Vanier, M.T.2    Saito, Y.3    Suzuki, K.4    Suzuki, K.5
  • 27
    • 0028795969 scopus 로고
    • Proliferative capacity of oligodendrocytes in the demyelinating twitcher spinal cord
    • Taniike, M., and Suzuki, K. (1995) Proliferative capacity of oligodendrocytes in the demyelinating twitcher spinal cord J. Neurosci. Res. 40, 325-332.
    • (1995) J. Neurosci. Res. , vol.40 , pp. 325-332
    • Taniike, M.1    Suzuki, K.2
  • 28
    • 0033065014 scopus 로고    scopus 로고
    • An apoptotic depletion of oligodendrocytes in the twitcher, a murine model of globoid cell leukodystrophy
    • Taniike, M, Mohri. I., Eguchi, N., Irikura, D., Urade, Y, Okada, S., and Suzuki, K. (1999) An apoptotic depletion of oligodendrocytes in the twitcher, a murine model of globoid cell leukodystrophy. J. Neuropathol Exp. Neurol. 58, 644-653.
    • (1999) J. Neuropathol. Exp. Neurol. , vol.58 , pp. 644-653
    • Taniike, M.1    Mohri, I.2    Eguchi, N.3    Irikura, D.4    Urade, Y.5    Okada, S.6    Suzuki, K.7
  • 29
    • 0034886801 scopus 로고    scopus 로고
    • Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH-1 cells
    • Tohyama, J, Matsuda, J., and Suzuki, K. (2001) Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH-1 cells. Neurochem. Res. 26, 667-671
    • (2001) Neurochem. Res. , vol.26 , pp. 667-671
    • Tohyama, J.1    Matsuda, J.2    Suzuki, K.3
  • 30
    • 0027378910 scopus 로고
    • MHC class II antigen expression and T-cell infiltration in the demyelinating CNS and PNS of the twitcher mouse
    • Ohno, M., Komiyama, A., Martin, P M., and Suzuki, K (1993) MHC class II antigen expression and T-cell infiltration in the demyelinating CNS and PNS of the twitcher mouse. Brain Res. 625, 186-196.
    • (1993) Brain Res. , vol.625 , pp. 186-196
    • Ohno, M.1    Komiyama, A.2    Martin, P.M.3    Suzuki, K.4
  • 32
    • 0027994601 scopus 로고
    • Absence of MHC class II molecules reduces CNS demyelination, microglial; macrophage infiltration, and twitching in murine globoid cell leukodystrophy
    • Matsushima, G. K., Taniike, M., Glimcher, L H., Grusby, M. J., Frelinger, J. A., Suzuki, K., and Ting, J. P. Y. (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    Taniike, M.2    Glimcher, L.H.3    Grusby, M.J.4    Frelinger, J.A.5    Suzuki, K.6    Ting, J.P.Y.7
  • 33
    • 0028340128 scopus 로고
    • Neuropathology of twitcher mice. Examination by histochemistry, immunohistochemistry, lectin histochemistry and Fourier transform infrared microspectroscopy
    • LeVine, S. M., Wetzel, D. L., and Eiler, A. J. (1994) Neuropathology of twitcher mice. Examination by histochemistry, immunohistochemistry, lectin histochemistry and Fourier transform infrared microspectroscopy. J. Develop. Neurosci. 12, 275-288.
    • (1994) J. Develop. Neurosci. , vol.12 , pp. 275-288
    • LeVine, S.M.1    Wetzel, D.L.2    Eiler, A.J.3
  • 34
    • 0030614992 scopus 로고    scopus 로고
    • IL-6 and TNFα expression in brains of twitcher, quaking and normal mice
    • LeVine, S. M., and Brown, D C. (1997) IL-6 and TNFα expression in brains of twitcher, quaking and normal mice. J. Neuroimmunol. 73, 47-56.
    • (1997) J. Neuroimmunol. , vol.73 , pp. 47-56
    • LeVine, S.M.1    Brown, D.C.2
  • 35
    • 0032588924 scopus 로고    scopus 로고
    • IL-6 deficiency causes enhanced pathology in twitcher (globoid cell leukodystrophy) mice
    • Pedchenko, T. V., and LeVine, S. M. (1999) IL-6 deficiency causes enhanced pathology in twitcher (globoid cell leukodystrophy) mice. Exp. Neurol. 158, 459-468.
    • (1999) Exp. Neurol. , vol.158 , pp. 459-468
    • Pedchenko, T.V.1    LeVine, S.M.2
  • 36
    • 0034597162 scopus 로고    scopus 로고
    • TNF-receptor I deficiency fails to alter the clinical and pathological course in mice with globoid cell leukodystrophy (twitcher mice) but affords protection following LPS challenge
    • Pedchenko, T. V., Broshteyn, I. G., and LeVine, S M. (2000) TNF-receptor I deficiency fails to alter the clinical and pathological course in mice with globoid cell leukodystrophy (twitcher mice) but affords protection following LPS challenge. J. Neuroimmunol. 110, 186-194.
    • (2000) J. Neuroimmunol. , vol.110 , pp. 186-194
    • Pedchenko, T.V.1    Broshteyn, I.G.2    LeVine, S.M.3
  • 37
    • 0034759421 scopus 로고    scopus 로고
    • Expression of immune-related molecules is downregulated in Twitcher mice following bone marrow transplantation
    • Wu, Y. P., McMahon, E. J., Matsuda, J., Suzuki, K., Matsushima, G. K., 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
  • 38
    • 0034650965 scopus 로고    scopus 로고
    • Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide
    • Ezoe, T., Vanier, M T., Oya, Y., Popko, B., Tohyama, J., Matsuda, J., Suzuki, K., and Suzuki, K. (2000) Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide. J. Neurosci. Res. 59, 170-178.
    • (2000) J. Neurosci. Res. , vol.59 , pp. 170-178
    • Ezoe, T.1    Vanier, M.T.2    Oya, Y.3    Popko, B.4    Tohyama, J.5    Matsuda, J.6    Suzuki, K.7    Suzuki, K.8
  • 40
    • 0035897409 scopus 로고    scopus 로고
    • Identification of a molecular target of psychosine and its role in globoid cell formation
    • Im, D. S, Heise, C. E., Nguyen, T., O'Dowd, B. F., and Lynch, K. R. (2001) Identification of a molecular target of psychosine and its role in globoid cell formation. J. Cell Biol 153, 429-434.
    • (2001) J. Cell Biol. , vol.153 , pp. 429-434
    • Im, D.S.1    Heise, C.E.2    Nguyen, T.3    O'Dowd, B.F.4    Lynch, K.R.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.