1. Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease
- Author
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Alexander Peden, Diane Ritchie, James W. Ironside, Mark Head, Helen Yull, Richard Bonshek, and Andrew B Tullo
- Subjects
Male ,Pathology ,medicine.medical_specialty ,PrPSc Proteins ,genetic structures ,animal diseases ,Blotting, Western ,Clinical Science - Scientific Reports ,Biology ,Creutzfeldt-Jakob Syndrome ,Retina ,Central nervous system disease ,Cellular and Molecular Neuroscience ,chemistry.chemical_compound ,Degenerative disease ,mental disorders ,medicine ,Humans ,Aged ,Paraffin Embedding ,Retinal ,medicine.disease ,Immunohistochemistry ,Virology ,Sensory Systems ,nervous system diseases ,Blot ,Ophthalmology ,medicine.anatomical_structure ,chemistry ,sense organs - Abstract
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.
- Published
- 2005