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Globoid cell leucodystrophy in polled Dorset sheep

D H Pritchard, D V Napthine, A J Sinclair

    Veterinary Pathology
    |July 1, 1980
    PubMed
    Summary
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    Globoid cell leucodystrophy, a rare genetic disorder, was diagnosed in Polled Dorset sheep. Affected sheep showed severe neurological signs due to a deficiency in the galactocerebrosidase enzyme.

    Area of Science:

    • Veterinary Neurology
    • Biochemistry
    • Genetics

    Background:

    • Globoid cell leucodystrophy (Krabbe's disease) is a severe lysosomal storage disorder affecting myelin.
    • This study investigates Krabbe's disease in a flock of Polled Dorset sheep.

    Observation:

    • Two sheep presented with hind limb incoordination and progressive tetraplegia.
    • Histopathology revealed myelin destruction, oligodendroglial loss, astrogliosis, and PAS-positive globoid cells in the white matter.

    Findings:

    • Enzyme analysis showed a deficiency of galactocerebrosidase (less than 6% of control mean) in the affected sheep's brain.
    • Activities of three other glycosidase enzymes were comparable between affected and unaffected sheep, indicating specificity of the deficiency.

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    Implications:

    • This diagnosis confirms globoid cell leucodystrophy in Polled Dorset sheep, highlighting a potential genetic susceptibility.
    • Understanding the biochemical basis (galactocerebrosidase deficiency) is crucial for potential diagnostic and management strategies in affected livestock.