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Vet Pathol 43:733-739 (2006)
© 2006 American College of Veterinary Pathologists

Abnormal Prion Accumulation Associated with Retinal Pathology in Experimentally Inoculated Scrapie-Affected Sheep

J. J. Greenlee, A. N. Hamir and M. H. West Greenlee

Virus and Prion Diseases of Livestock Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, Ames, IA (JJG, ANH), and the Department of Biomedical Sciences, Interdepartmental Neuroscience Program, Bioinformatics and Computational Biology Program, Iowa State University, Ames, IA (MHWG)

The purpose of this study was to characterize the patterns of PrPSc immunoreactivity in the retinae of scrapie-affected sheep and to determine the extent of retinal pathology as indicated by glial fibrillary acidic protein immunoreactivity (GFAP-IR) of Müller glia. Sections from the retina of 13 experimentally inoculated scrapie-affected and 2 negative control sheep were examined with immunohistochemical staining for PrPSc, GFAP, and PrPSc/GFAP double staining. GFAP-IR of Müller glia is suggestive of retinal pathology in the absence of morphologic abnormality detected by light microscopy. Sheep with the least amount of PrPSc in the retina have multifocal punctate aggregates of prion staining in the outer half of the inner plexiform layer and rarely in the outer plexiform layer. In these retinae, GFAP-IR is not localized with prion accumulation, but rather is present in moderate numbers of Müller glia throughout the sections of retina examined. The majority of sheep with retinal accumulation of PrPSc have intense, diffuse PrPSc staining in both plexiform layers, with immunoreactivity in the cytoplasm of multiple ganglion cells and lesser amounts in the optic fiber layer and between nuclei in nuclear layers. This intense PrPSc immunoreactivity is associated with diffuse, intense GFAP-IR that extends from the inner limiting membrane to the outer limiting membrane. This is the first report of a prion disease in a natural host that describes the accumulation of PrPSc in retina associated with retinal pathology in the absence of overt morphologic changes indicative of retinal degeneration.

Key words: Glial fibrillary acidic protein; immunohistochemistry; prion diseases; retina; scrapie; sheep; spongiform encephalopathy.

Request reprints from Dr. J J Greenlee, National Animal Disease Center, 2300 Dayton Ave., Ames, IA 50010






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