@article {Jolly:1  October 2002:0048-0169:199,
	author = "Jolly R.D.",
	author = "Arthur D.G.",
	author = "Kay G.W.",
	author = "Palmer D.N.",
	title = "Neuronal ceroid-lipofuscinosis in Borderdale sheep",
	journal = "New Zealand Veterinary Journal",
	volume = "50",
	year = "1  October 2002",
	abstract = "<P></P>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. <P></P>METHODS: Formalin-fixed tissues were processed into paraffin wax and epoxy resin for light and electron microscopy of variously stained sections. <P></P>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. <P></P>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. <P></P>KEY WORDS: Sheep, neuronal ceroid-lipofuscinosis, subunit-c, mitochondrial ATP synthase",
	pages = "199-202(4)",
	url = "http://www.ingentaconnect.com/content/nzva/nzvj/2002/00000050/00000005/art00006"
}