NR AEWX
AU Grigoriev,V.B.; Escaig-Haye,F.; Streichenberger,N.; Kopp,N.; Langeveld,J.P.M.; Brown,P.; Fournier,J.G.
TI Submicroscopic immunodetection of PrP in the brain of a patient with a new-variant of Creutzfeldt-Jakob disease
QU Neuroscience Letters 1999 Apr 2; 264(1-3): 57-60
PT journal article
AB We analyzed the distribution and organization of the pathological prion protein isoform (PrPsc) in the brain of new variant Creutzfeldt-Jakob disease using a sensitive post-embedding immunogold electron microscopy method. On methacrylate semithin sections, silver-PrP staining showed florid plaques, containing microvacuoles. It also revealed scattered granular and perivacuolar deposits. At the electron microscope level, plaque PrP-gold labeling was associated with filaments and flocculent amorphous material sometimes observed inside microvacuoles, considered as degenerative neurites. Outside the plaques, PrP-gold labeling was predominantly found over flocculent amorphous material and the presynaptic domain of synapses. Some lysosome-like organelles seen in the neuron perikaryon, were also found to be PrP-immunoreactive. These results are consistent with the hypothesis that the synapse is a privileged target in prion disease.
MH Adult; Brain/*metabolism/ultrastructure; Case Report; Creutzfeldt-Jakob Syndrome/*genetics/*metabolism/pathology; Human; Immunohistochemistry; Microscopy, Immunoelectron; Organelles/metabolism/ultrastructure; PrPsc Proteins/*metabolism; Presynaptic Terminals/metabolism/ultrastructure; Support, Non-U.S. Gov't; Tissue Distribution/physiology; Vacuoles/metabolism/ultrastructure
AD Unite 523 INSERM, Institut de Myologie, Hopital Pitie-Salpetriere, Paris, France.
SP englisch
PO Irland