NR AVIT
AU Greenlee,J.J.; Hamir,A.N.; West Greenlee,M.H.
TI Abnormal prion accumulation associated with retinal pathology in experimentally inoculated scrapie-affected sheep
QU Veterinary Pathology 2006 Sep; 43(5): 733-9
PT journal article
AB 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 Muller 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 Muller 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 Muller 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.
IN
Bei experimentell mit Scrapie infizierten Schafen ist PrPsc zuerst in der näher am Sehnerv gelegenen inneren plexiformen Schicht und erst später in der äußeren plexiformen Schicht der Retina zu finden. Beide bestehen hauptsächlich aus Synapsen.
MH Animals; Glial Fibrillary Acidic Protein/metabolism; Neuroglia/metabolism; Prions/*metabolism; Retina/*metabolism/*pathology; Retinal Diseases/pathology/*veterinary; Scrapie/*metabolism/*pathology; Sheep; Up-Regulation
AD Virus and Prion Diseases of Livestock Research Unit, National Animal Disease Center, USDA Agricultural Research Service, 2300 Dayton Ave., Ames, IA 50010, USA
SP englisch
PO USA