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Volumn 14, Issue 3, 1996, Pages 348-352

Mice lacking both subunits of lysosomal β-hexosaminidase display gangliosidosis and mucopolysaccharidosis

Author keywords

[No Author keywords available]

Indexed keywords

BETA N ACETYLHEXOSAMINIDASE; GANGLIOSIDE; GLYCOSAMINOGLYCAN; ISOENZYME; SPHINGOLIPID;

EID: 16144368388     PISSN: 10614036     EISSN: 15461718     Source Type: Journal    
DOI: 10.1038/ng1196-348     Document Type: Letter
Times cited : (178)

References (12)
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    • Yamanaka, S. et al. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease. Proc. Natl. Acad. Sci. USA 91, 9975-9979 (1994).
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    • Yamanaka, S.1
  • 2
    • 0029113867 scopus 로고
    • Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism
    • Sango, K. et al. Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nature Genet. 11, 170-176(1995).
    • (1995) Nature Genet , vol.11 , pp. 170-176
    • Sango, K.1
  • 3
    • 0015358976 scopus 로고
    • Mucopolysaccharide storage in organs of a patient with Sandhoff's disease
    • Applegarth, D.A. & Bozoian, G. Mucopolysaccharide storage in organs of a patient with Sandhoff's disease. Clin. Chim. Acta 39, 269-271 (1972).
    • (1972) Clin. Chim. Acta , vol.39 , pp. 269-271
    • Applegarth, D.A.1    Bozoian, G.2
  • 6
    • 0028909644 scopus 로고
    • Neuropathology of mice with targeted disruption of Hexa gene, a model of Tay-Sachs disease
    • Taniike, M. et al. Neuropathology of mice with targeted disruption of Hexa gene, a model of Tay-Sachs disease. Acta Neuropathol. 89, 296-304 (1995).
    • (1995) Acta Neuropathol , vol.89 , pp. 296-304
    • Taniike, M.1
  • 7
    • 0030032179 scopus 로고    scopus 로고
    • Fluorophore-assisted carbohydrate electrophoresis in the separation, analysis, and sequencing of carbohydrates
    • Starr, C.M., Masada, R.I., Hague, C, Skop, E. & Klock, J.C. Fluorophore-assisted carbohydrate electrophoresis in the separation, analysis, and sequencing of carbohydrates. J. Chromatogr. A 720, 295-321 (1996).
    • (1996) J. Chromatogr. A , vol.720 , pp. 295-321
    • Starr, C.M.1    Masada, R.I.2    Hague, C.3    Skop, E.4    Klock, J.C.5
  • 8
    • 0014293028 scopus 로고
    • The defect in Hurler's and Hunter's syndromes: Faulty degradation of mucopolysaccharide
    • Fratantoni, J.C., Hall, C.W. & Neufeld, E.F. The defect in Hurler's and Hunter's syndromes: Faulty degradation of mucopolysaccharide. Proc. Natl. Acad. Sci. USA 60, 699-706 (1968).
    • (1968) Proc. Natl. Acad. Sci. USA , vol.60 , pp. 699-706
    • Fratantoni, J.C.1    Hall, C.W.2    Neufeld, E.F.3
  • 9
    • 0016286842 scopus 로고
    • Sandhoff disease: Defective glycosaminoglycan catabolism in cultured fibroblasts and its correction by β-N-acetylhexosaminidase
    • Cantz, M. & Kresse, H. Sandhoff disease: defective glycosaminoglycan catabolism in cultured fibroblasts and its correction by β-N-acetylhexosaminidase. Eur. J. Biochem. 47, 581-590 (1974).
    • (1974) Eur. J. Biochem , vol.47 , pp. 581-590
    • Cantz, M.1    Kresse, H.2
  • 11
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    • A murine model of mucopolysaccharidosis VII: Gross and microscopic findings in beta-glucuronidase-deficient mice
    • Vogler, C. et al. A murine model of mucopolysaccharidosis VII: Gross and microscopic findings in beta-glucuronidase-deficient mice. Am. J. Pathol. 136, 207-217 (1990).
    • (1990) Am. J. Pathol , vol.136 , pp. 207-217
    • Vogler, C.1
  • 12
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    • β-N-Acetylhexosaminidase active on dermatan sulfate
    • Singh, J., Coppa, G.V. & DiFerrante, N. β-N-Acetylhexosaminidase active on dermatan sulfate. Enzyme 19, 15-23 (1975).
    • (1975) Enzyme , vol.19 , pp. 15-23
    • Singh, J.1    Coppa, G.V.2    Diferrante, N.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.