Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease.
BACKGROUND
Involvement of the eye has been reported in patients with variant Creutzfeldt-Jakob disease (vCJD), but there is disagreement on whether retinal involvement occurs in sporadic Creutzfeldt-Jakob disease (sCJD).


METHODS
Western blotting, paraffin embedded tissue blotting, and immunohistochemistry were used to test whether the abnormal form of the prion protein (PrPSc) accumulates to detectable levels in the eye in a case of the most common subtype of sCJD (MM1).


RESULTS
Low levels of PrPSc were detectable in the retina, localised to the plexiform layers of the central retina. PrPSc was not detectable in other ocular tissues.


CONCLUSIONS
The abnormal form of the prion protein is present in the retina in the most common sCJD subtype (MM1), albeit at levels lower than those found previously in vCJD and in sCJD of the VV2 subtype.
Head
M W
MW
National CJD Surveillance Unit, Bryan Matthews Building, Western General Hospital, University of Edinburgh EH4 2XU, UK. m.w.head@ed.ac.uk
Peden
A H
AH
Yull
H M
HM
Ritchie
D L
DL
Bonshek
R E
RE
Tullo
A B
AB
Ironside
J W
JW
D000368:Aged; D015153:Blotting, Western / Q000379:methods; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D016612:Paraffin Embedding; D018620:PrPSc Proteins / Q000032:analysis*; D012160:Retina / Q000737:chemistry*
D018620:PrPSc Proteins