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Characteristic inclusions in the kidney of canine globoid cell leukodystrophy

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Summary

The kidney of a 7-month-old male Cairn terrier with globoid cell leukodystrophy (GLD) was investigated with light and electron microscopes. A few tubular epithelial cells in the inner medulla as well as some exfoliated cells in the lumina revealed PAS-positive cytoplasm in which needle-like structures were to be seen on occasion. At the ultrastructural level, characteristic inclusions of GLD were found in these cells. This observation indicates that in addition to our previous report in the kidney of murine GLD (Takahashi et al. 1984), kidney in canine GLD also is a site of abnormal storage of galactosylceramide, although so far no morphological or biochemical evidence of galactosylceramide storage was demonstrated in human GLD

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References

  • Andrews JM, Cancilla PA (1970) Cytoplasmic inclusions in globoid cell leukodystrophy. Arch Pathol 89:53–55

    Google Scholar 

  • Andrews JM, Menkes JH (1970) Ultrastructure of experimentally produced globoid cells in the rat. Exp Neurol 29:483–493

    Google Scholar 

  • Austin JH (1968) Globoid (Krabbe) leukodystrophy. In: Minkler J (ed) Pathology of the nervous system, vol 1. Mc Graw-Hill, New York, pp 843–858

    Google Scholar 

  • Anzil AP, Blinzinger K, Mehraein P, Dorn G, Neuhauser G (1972) Cytoplasmic inclusions in a child affected with Krabbe's disease (globoid cell leukodystrophy) and in the rabbit injected with galactocerebrosides. J Neuropathol Exp Neurol 31:370–388

    Google Scholar 

  • Bishoff A, Urich J (1969) Peripheral neuropathy in globoid cell leukodystrophy (Krabbe's) disease: Ultrastructure and histochemical findings. Brain 92:861–870

    Google Scholar 

  • Duchen LW, Eicher EM, Jacobs J, Scaravilli F, Texeira F (1980) Hereditary leukodystrophy in the mouse: The new mutant twitcher. Brain 103:695–710

    Google Scholar 

  • Frankhauser R, Luginbühl H, Hartley WJ (1963) Leukodystrophie vom Typus Krabbe beim Hund. Schweiz Arch Tierheilkd 105:198–207

    Google Scholar 

  • Fletcher TF, Lee DG, Hammer RF (1971) Ultrastructural features of globoid cell leukodystrophy in the dog. Am J Vet Res 32:177–181

    Google Scholar 

  • Fletcher TF, Jessen CR, Bender AP (1977) Quantitative evaluations of spinal cord lesions in canine globoid leukodystrophy. J Neuropathol Exp Neurol 36:84–99

    Google Scholar 

  • Ida H, Umezawa F, Kasai E, Eto Y, Mackawa K (1982) An accumulation of galactocerebroside in kidney from mouse globoid cell leukodystrophy (twitcher). Biochem Biophys Res Commun 109:634–638

    Google Scholar 

  • Igisu H, Takahashi H, Suzuki K, Suzuki K (1983) Abnormal accumulation of galactosylceramide in the kidney of the twitcher mouse. Biochem Biophys Res Commun 110:940–944

    Google Scholar 

  • Johnson KH (1970) Globoid leukodystrophy in the cat. J Am Vet Med Assoc 157:2057–2064

    Google Scholar 

  • Jortner BS, Jonas AM (1968) The neuropathology of globoid cell leukodystrophy in the dog. Acta Neuropathol (Berl) 10:171–182

    Google Scholar 

  • Kobayashi T, Yamanaka T, Jacobs JM Texeira F, Suzuki K (1980) The twitcher mouse: An enzymatically authentic model of human globoid cell leukodystrophy (Krabbe's disease). Brain Res 202:479–483

    Google Scholar 

  • Makita A (1964) Biochemistry of organ glycolipid. II. Isolation of human kidney glycolipid. J Biochem 55:269–276

    Google Scholar 

  • Martenson E (1966) Neutral glycolipids of human kidney: Isolation, identification, and fatty acid composition. Biochim Biophys Acta 116:296–308

    Google Scholar 

  • Mei Liu H (1970) Ultrastructure of globoid leukodystrophy (Krabbe's disease) with reference to the origin of globoid cells. J Neuropathol Exp Neurol 29:441–462

    Google Scholar 

  • Narasimhan R, Murray RK (1979) Neutral glycolipids and gangliosides of human lung and lung tumors. Biochem J 179:199–211

    Google Scholar 

  • Nilsson O, Svennerholm L (1982) Characterization and quantitative determination of gangliosides and neutral glycolipids in human liver. J Lipid Res 23:327–334

    Google Scholar 

  • Norton W, Cammer W (1984) Isolation and characterization of myelin. In: Morell P (ed) Myelin. Plenum Press, New York, pp 147–180

    Google Scholar 

  • Okeda R, Suzuki Y, Horiguchi S, Fujii T (1979) Fetal globoid cell leukodystrophy in one of twins. Acta Neuropathol (Berl) 47:151–154

    Google Scholar 

  • Pritchard DH, Napthina DV, Sinclair AJ (1980) Globoid cell leukodystrophy in polled dorset sheep. Vet Pathol 17:399–405

    Google Scholar 

  • Schochet SS Jr, Hardman JM, Lampert PW, Earle KM (1969) Krabbe's disease (globoid leukodystrophy). Electron-microscopic observations. Arch Pathol 88:305–313

    Google Scholar 

  • Suzuki K (1970) Ultrastructural study of experimental globoid cells. Lab Invest 23:612–619

    Google Scholar 

  • Suzuki K (1971) Renal cerebroside in globoid cell leukodystrophy (Krabbe's disease). Lipids 6:433–436

    Google Scholar 

  • Suzuki K, Grover WD (1970) Krabbe's leukodystrophy (globoid cell leukodystrophy). An ultrastructural study. Arch Neurol 22:385–396

    Google Scholar 

  • Suzuki K, Suzuki Y (1983) Galactosylceramide lipidosis: Globoid cell leukodystrophy (Krabbe's disease). In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) Metabolic basis of inherited disease. McGraw-Hill, New York, pp 857–880

    Google Scholar 

  • Suzuki K, Suzuki K (1985) Genetic galactosylceramidase deficiency (globoid cell leukodystrophy, Krabbe disease) in different mammalian species. Neurochem Pathol 3:53–68

    Google Scholar 

  • Takahashi H, Igisu H, Suzuki K, Suzuki K (1983) Murine globoid cell leukodystrophy (the twitcher mouse). The presence of characteristic inclusions in the kidney and lymph nodes. Am J Pathol 112:147–154

    Google Scholar 

  • Takahashi H, Igisu H, Suzuki K, Suzuki K (1984) Murine globoid cell leukodystrophy: The twitcher. An ultrastructural study of the kidney. Lab Invest 50:42–50

    Google Scholar 

  • Yajima K, Fletcher TF, Suzuki K (1977) Ultrastructural studies on canine globoid cell leukodystrophy. J Neurol Sci 33:179–197

    Google Scholar 

  • Yunis EJ, Lee RE (1969) The ultrastructure of globoid (Krabbe) leukodystrophy. Lab. Invest. 21:415–419

    Google Scholar 

  • Yunis EJ, Lee RE (1976) The morphologic similarities of human and canine globoid cell leukodystrophy: Thin section and freeze-fracture studies. Am J Pathol 85:99–114

    Google Scholar 

  • Zaki FA, Kay WJ (1973) Globoid cell leukodystrophy in a miniature poodle. J Am Vet Med Assoc 163:248–250

    Google Scholar 

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Authors and Affiliations

  1. Depts. of Pathology (Neuropathology) and Neuroscience, Albert Einstein College of Medicine, 10461, Bronx, NY, USA

    K. Suzuki

  2. Rose F. Kennedy Center for Research in Mental Retardation and Human Development, Albert Einstein College of Medicine, 10461, Bronx, NY, USA

    K. Suzuki

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  1. K. Suzuki
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Supported in part by research grants NS-03356, NS-10803, and HD-07098 from NINCDS, USPHS.

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Suzuki, K. Characteristic inclusions in the kidney of canine globoid cell leukodystrophy. Acta Neuropathol 69, 33–37 (1986). https://doi.org/10.1007/BF00687036

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  • DOI: https://doi.org/10.1007/BF00687036

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