The Morphologic Similarities of Human and Canine Globoid Leukodystrophy. Thin Section and Freeze-fracture Studies

Overview

Journal Am J Pathol

Publisher Elsevier

Specialty Pathology

Date 1976 Oct 1

PMID 970445

Citations 7

Authors

E J Yunis

R E Lee

Affiliations

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Abstract

Human and canine globoid leukodystrophy are shown to be indistinguishable morphologically. Both have characteristic deposits with polygonal cross-sectional profiles in addition to twisted ribbon-like structures. The specificity of the deposits is emphasized, and their laminated nature is corroborated by the freeze-fracture studies. Their apparently hollow profiles in cross section are thought to be produced by the tight arrangement of the bilayers that prevent penetration of the stain.

Citing Articles

Fetal Krabbe leukodystrophy. A morphologic study of two cases.

Martin J, Leroy J, Ceuterick C, Libert J, DODINVAL P, Martin L Acta Neuropathol. 1981; 53(2):87-91.

PMID: 7211207 DOI: 10.1007/BF00689987.

A histochemical study of the enzyme profile of Krabbe's cells.

Elleder M Acta Neuropathol. 1983; 60(1-2):156-8.

PMID: 6880619 DOI: 10.1007/BF00685363.

Demyelination in the spinal cord of murine globoid cell leukodystrophy (the twitcher mouse).

Takahashi H, Suzuki K Acta Neuropathol. 1984; 62(4):298-308.

PMID: 6730907 DOI: 10.1007/BF00687612.

Globoid cell leukodystrophy: specialized contact of globoid cell with astrocyte in the brain of twitcher mouse.

Takahashi H, Suzuki K Acta Neuropathol. 1982; 58(4):237-42.

PMID: 6297224 DOI: 10.1007/BF00688603.

Characteristic inclusions in the kidney of canine globoid cell leukodystrophy.

Suzuki K Acta Neuropathol. 1986; 69(1-2):33-7.

PMID: 3008490 DOI: 10.1007/BF00687036.

References

Lee R, Balcerzak S, WESTERMAN M . Gaucher's disease. A morphologic study and measurements of iron metabolism. Am J Med. 1967; 42(6):891-8. DOI: 10.1016/0002-9343(67)90070-8. View

Schaumburg H, Powers J, Raine C, Suzuki K, Richardson Jr E . Adrenoleukodystrophy. A clinical and pathological study of 17 cases. Arch Neurol. 1975; 32(9):577-91. DOI: 10.1001/archneur.1975.00490510033001. View

SCHOCHET Jr S, Hardman J, LAMPERT P, EARLE K . Krabbe's disease (globoid leukodystrophy). Electron microscopic observations. Arch Pathol. 1969; 88(3):305-13. View

Andrews J, Cancilla P . Cytoplasmic inclusions in human globoid cell leukodystrophy. Krabbe's disease. Arch Pathol. 1970; 89(1):53-5. View

Steere R . Freeze-etching and direct observation of freezing damage. Cryobiology. 1969; 6(3):137-50. DOI: 10.1016/s0011-2240(69)80344-5. View

Yunis E, Lee R . Tubules of globoid leukodystrophy: a right-handed helix. Science. 1970; 169(3940):64-6. DOI: 10.1126/science.169.3940.64. View

Liu H . Ultrastructure of globoid leukodystrophy (Krabbe's disease) with reference to the origin of globoid cells. J Neuropathol Exp Neurol. 1970; 29(3):441-62. DOI: 10.1097/00005072-197007000-00008. View

Suzuki K, Suzuki Y . Globoid cell leucodystrophy (Krabbe's disease): deficiency of galactocerebroside beta-galactosidase. Proc Natl Acad Sci U S A. 1970; 66(2):302-9. PMC: 283044. DOI: 10.1073/pnas.66.2.302. View

Suzuki K . Ultrastructural study of experimental globoid cells. Lab Invest. 1970; 23(6):612-9. View

10.

Suzuki Y, Suzuki K . Krabbe's globoid cell leukodystrophy: deficiency of glactocerebrosidase in serum, leukocytes, and fibroblasts. Science. 1971; 171(3966):73-5. DOI: 10.1126/science.171.3966.73. View

11.

FLETCHER T, Lee D, Hammer R . Ultrastructural features of globoid-cell leukodystrophy in the dog. Am J Vet Res. 1971; 32(1):177-81. View

12.

Yunis E, Lee R . Further observations on the fine structure of globoid leukodystrophy. Peripheral neuropathy and optic nerve involvement. Hum Pathol. 1972; 3(3):371-88. DOI: 10.1016/s0046-8177(72)80038-8. View

13.

Lee R, Worthington C, Glew R . The bilayer nature of deposits occurring in Gaucher's disease. Arch Biochem Biophys. 1973; 159(1):259-66. DOI: 10.1016/0003-9861(73)90452-9. View

14.

Suzuki Y, Miyatake T, FLETCHER T, Suzuki K . Glycosphingolipid beta-galactosidases. 3. Canine form of globoid cell leukodystrophy; comparison with the human disease. J Biol Chem. 1974; 249(7):2109-12. View

15.

Miyatake T, Suzuki K . Additional deficiency of psychosine galactosidase in globoid cell leukodystrophy: an implication to enzyme replacement therapy. Birth Defects Orig Artic Ser. 1973; 9(2):136-40. View

16.

Vanier M, Svennerholm L . Chemical pathology of Krabbe's disease. III. Ceramide-hexosides and gangliosides of brain. Acta Paediatr Scand. 1975; 64(4):641-8. DOI: 10.1111/j.1651-2227.1975.tb03896.x. View

17.

Steere R . Freeze-etching simplified. Cryobiology. 1969; 5(5):306-23. DOI: 10.1016/s0011-2240(69)80459-1. View