Brain Copper Elevation and Neurological Changes in North Ronaldsay Sheep: a Model for Neurodegenerative Disease?

SummaryThis study in North Ronaldsay (NR) sheep showed that copper was elevated in both the liver and brain of older animals and that the elevation in these two sites was highly correlated. The accumulation of copper in the liver culminated in chronic active hepatitis. Evidence of tissue damage in the brain was equivocal, but the astrocytes showed strong immunoreactivity for metallothionein. The study suggested that the blood–brain barrier of NR sheep possesses unusual features in respect of the import of copper into the brain, and that NR sheep may provide a useful animal model for the investigation of brain copper homeostasis.