NR AVLJ
AU Moroncini,G.; Mangieri,M.; Morbin,M.; Mazzoleni,G.; Ghetti,B.; Gabrielli,A.; Williamson,R.A.; Giaccone,G.; Tagliavini,F.
TI Pathologic prion protein is specifically recognized in situ by a novel PrP conformational antibody
QU Neurobiology of Disease 2006 Sep; 23(3): 717-24
PT journal article
AB Prion diseases are characterized by the accumulation in the brain of abnormal conformers (PrPsc) of the cellular prion protein (PrPc). PrPsc immunohistochemistry, currently based on antibodies non-distinguishing between PrPc and PrPsc, requires pre-treatments of histological sections to eliminate PrPc and to denature PrPsc. We employed the PrPsc-specific antibody 89-112 PrP motif-grafted IgG on mildly fixed, untreated brain sections from several cases of human prion diseases. The results confirmed specific binding of IgG 89-112 to a structural determinant found exclusively on native disease-associated PrP conformations and lost following tissue denaturation or cross-linking fixation. Importantly, IgG 89-112 demonstrated no reactivity with normal brain tissue or with amyloid deposits in Alzheimer disease brain sections. Thus, immunohistochemical detection of native PrPsc deposits was obtained by means of a PrPsc-specific antibody. Such unique reagent may have many applications in the study of prion biology and in the diagnosis and prevention of prion diseases.
MH Amino Acid Motifs/immunology; Antibodies/chemistry/diagnostic use/*immunology; Antibody Specificity/*immunology; Artifacts; Brain/*immunology/metabolism/physiopathology; Cross Reactions/immunology; Humans; Immunohistochemistry/*methods/trends; PrPsc Proteins/analysis/*immunology/metabolism; Predictive Value of Tests; Prion Diseases/*diagnosis/immunology/physiopathology; Protein Conformation; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Senile Plaques/immunology
AD Dipartimento di Scienze Mediche e Chirurgiche, Sezione di Clinica Medica, Universita Politecnica delle Marche, Polo Didattico, Via Tronto 10, 60020 Ancona, Italy. g.moroncini@univpm.it
SP englisch
PO USA