Toluidine blue-O staining of prion protein deposits.
Prion diseases or transmissible spongiform encephalopathies are a group of fatal neurodegenerative diseases caused by an abnormal form of prion protein (PrP(sc)). In this study, we developed a sensitive histochemical detection of PrP(sc) deposits in a Gertsmann-Sträussler-Scheinker disease (GSS) patient using toluidine blue-O staining, a specific reagent to stain mucins and mucopolysaccharides. Detection of prion deposits correlated with immunohistochemistry using anti-prion antibodies. Control assays were performed using amyloid-beta (Abeta) plaques from Alzheimer's disease (AD) brains. Our results demonstrated that toluidine blue-O staining allowed to recognize 69.1+/-2.6% of the total plaques recognized by the anti-prion antibody. Furthermore, in the 15 studied brain regions from the GSS patient, toluidine blue-O revealed the same recognition pattern as anti-prion labeling. Toluidine blue-O stained specifically the prion deposits but not the Abeta plaques in AD brains. The specificity of the technique was confirmed in a Creutzfeldt-Jakob disease brain. This method opens several possibilities for postmortem diagnoses. Our results also suggest the relevance of specific post-translational modifications of PrP(sc), identified by toluidine blue-O, that might participate in the transformation of PrP(c) to PrP(sc).
Sánchez
A
A
Departamento de Neuropatología, Instituto Nacional de Neurología, Insurgentes Sur 3877, La Fama, 14269 Mexico, DF.
Guzmán
A
A
Ortiz
A
A
Rembao
D
D
Espinosa
B
B
Zenteno
E
E
Guevara
J
J
D000368:Aged; D000544:Alzheimer Disease / Q000378:metabolism / Q000473:pathology; D016229:Amyloid beta-Peptides / Q000032:analysis / Q000378:metabolism; D001923:Brain Chemistry; D004396:Coloring Agents; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism / Q000473:pathology; D019084:Fluorescent Antibody Technique, Indirect; D016098:Gerstmann-Straussler-Scheinker Disease / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D008875:Middle Aged; D018620:PrPSc Proteins / Q000032:analysis / Q000378:metabolism*; D012680:Sensitivity and Specificity; D013194:Staining and Labeling; D014048:Tolonium Chloride
D016229:Amyloid beta-Peptides; D004396:Coloring Agents; D018620:PrPSc Proteins; D014048:Tolonium Chloride