PMID- 16836987
OWN - NLM
STAT- MEDLINE
DCOM- 20061206
LR  - 20060828
IS  - 0006-8993 (Print)
IS  - 0006-8993 (Linking)
VI  - 1108
IP  - 1
DP  - 2006 Sep 7
TI  - Pathological findings in retina and visual pathways associated to natural Scrapie 
      in sheep.
PG  - 188-94
AB  - This work represents a comprehensive pathological description of the retina and 
      visual pathways in naturally affected Scrapie sheep. Twenty naturally affected 
      Scrapie sheep and 6 matched controls were used. Eyes, optic nerves and brain from 
      each animal were fixed and histologically processed using hematoxylin-eosin, 
      followed by immunohistochemical staining for prion protein (PrPsc) and glial 
      fibrillar acidic protein (GFAP). Retinal histopathological changes were observed 
      in only 7 clinically affected animals and mainly consisted of loss of outer 
      limitant layer definition, outer plexiform layer atrophy, disorganization and 
      loss of nuclei in both nuclear layers, and Muller glia hypertrophy. PrPsc was 
      detected in the retina of 19 of the 20 sheep and characterized by a disseminated 
      granular deposit across layers and intraneuronally in ganglion cells. The inner 
      plexiform and the ganglion cell layers were the structures most severely affected 
      by PrPsc deposits. PrPsc exhibited a tendency to spread from these two layers to 
      the others. A marked increase in the number and intensity of GFAP-expressing 
      Muller cells was observed in the clinical stage, especially at the terminal stage 
      of the disease. Spongiosis and PrPsc were detected within the visual pathways at 
      the preclinical stage, their values increasing during the course of the disease 
      but varying between the areas examined. PrPsc was detected in only 3 optic 
      nerves. The results suggest that the presence of PrPsc in the retina correlates 
      with disease progression during the preclinical and clinical stages, perhaps 
      using the inner plexiform layer as a first entry site and diffusing from the 
      brain using a centrifugal model.
FAU - Hortells, Paloma
AU  - Hortells P
AD  - Faculty of Veterinary, University of Zaragoza, Miguel Servet 177, 50013 Zaragoza, 
      Spain.
FAU - Monzon, Marta
AU  - Monzon M
FAU - Monleon, Eva
AU  - Monleon E
FAU - Acin, Cristina
AU  - Acin C
FAU - Vargas, Antonia
AU  - Vargas A
FAU - Bolea, Rosa
AU  - Bolea R
FAU - Lujan, Lluis
AU  - Lujan L
FAU - Badiola, Juan Jose
AU  - Badiola JJ
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20060711
PL  - Netherlands
TA  - Brain Res
JT  - Brain research
JID - 0045503
RN  - 0 (Glial Fibrillary Acidic Protein)
RN  - 0 (PrPSc Proteins)
SB  - IM
MH  - Animals
MH  - Brain/metabolism/pathology/physiopathology
MH  - Brain Stem/metabolism/pathology/physiopathology
MH  - Disease Progression
MH  - Glial Fibrillary Acidic Protein/metabolism
MH  - Immunohistochemistry
MH  - Optic Nerve/metabolism/pathology/physiopathology
MH  - PrPSc Proteins/metabolism
MH  - Retina/metabolism/*pathology/physiopathology
MH  - Retinal Ganglion Cells/metabolism/pathology
MH  - Scrapie/*diagnosis/physiopathology
MH  - Sheep
MH  - Visual Pathways/metabolism/*pathology/physiopathology
EDAT- 2006/07/14 09:00
MHDA- 2006/12/09 09:00
CRDT- 2006/07/14 09:00
PHST- 2005/11/18 00:00 [received]
PHST- 2006/06/05 00:00 [revised]
PHST- 2006/06/05 00:00 [accepted]
PHST- 2006/07/14 09:00 [pubmed]
PHST- 2006/12/09 09:00 [medline]
PHST- 2006/07/14 09:00 [entrez]
AID - S0006-8993(06)01714-8 [pii]
AID - 10.1016/j.brainres.2006.06.021 [doi]
PST - ppublish
SO  - Brain Res. 2006 Sep 7;1108(1):188-94. doi: 10.1016/j.brainres.2006.06.021. Epub 
      2006 Jul 11.