title: Neuronal ceroid-lipofuscinosis in Borderdale sheep.
abstract: OBJECTIVE
  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.
authors: Institute of Veterinary Animal and Biomedical Sciences, Massey University, Private Bag 11222, Palmerston North, New Zealand. R.D.Jolly@massey.ac.nz
authors: R D
authors: RD
authors: Jolly
authors: D G
authors: DG
authors: Arthur
authors: G W
authors: GW
authors: Kay
authors: D N
authors: DN
authors: Palmer