IngentaConnect Neuronal ceroid-lipofuscinosis in Borderdale sheep

Authors: Jolly R.D.; Arthur D.G.; Kay G.W.; Palmer D.N.

Source: New Zealand Veterinary Journal, Volume 50, Number 5, 1 October 2002 , pp. 199-202(4)

Publisher: New Zealand Veterinary Association

Abstract:

AIM: To describe the gross and histological lesions of a neurological disease in Borderdale sheep characterised clinically by blindness and circling, as a basis to its classification.

METHODS: Formalin-fixed tissues were processed into paraffin wax and epoxy resin for light and electron microscopy of variously stained sections.

RESULTS: Lesions were those of a lysosomal storage disease with severe neurodegeneration of the cerebral cortex. The staining reactions, autofluorescence and ultrastructure of storage material allowed the diagnosis of neuronal ceroid-lipofuscinosis associated with the accumulation of subunit-c of mitochondrial ATP synthase.

CONCLUSIONS: The severity of neurodegeneration and minor differences in the ultrastructure of storage material implied that this was a different disease from other forms of ovine ceroid-lipofuscinosis that accumulate subunit-c of mitochondrial ATP synthase. An autosomal recessive mode of inheritance is considered probable. Although of only minor economic importance, this disease may be important to research into the group of ceroid-lipofuscinoses as a whole.

KEY WORDS: Sheep, neuronal ceroid-lipofuscinosis, subunit-c, mitochondrial ATP synthase

The full text electronic article is available for purchase. You will be able to download the full text electronic article after payment.

$25.00 plus tax