NR AAUY
AU Askanas,V.; Bilak,M.; Engel,W.K.; Leclerc,A.; Tome,F.
TI Prion protein is strongly immunolocalized at the postsynaptic domain of human normal neuromuscular junctions
QU Neuroscience Letters 1993 Sep 3; 159(1-2): 111-4
PT journal article
AB Using three well-characterized polyclonal and monoclonal antibodies against prion protein (PrP), we demonstrated a strong concentration of PrP at human neuromuscular junctions (NMJs). Applying double and triple fluorescence-labeling, we found that PrP immunoreactivity exactly co-localized with alpha-bungarotoxin (alpha-BT) identified acetylcholine receptors, as well as with the high junctional concentrations of beta-amyloid precursor protein, beta-amyloid protein, desmin, ubiquitin and dystrophin. Therefore, PrP was considered to be located on the postsynaptic muscle membrane. At all NMJs identified by bound alpha-BT, strong PrP immunoreactivity was obtained with all PrP antibodies. This appears to be the first demonstration of PrP concentrated at human NMJs.
IN Durch immunologische Mehrfachmarkierung wurde gezeigt, dass das normale Prionprotein in größeren Mengen in den postsynaptischen Membranen der motorischen Endplatte vorkommt.
MH Antibodies, Monoclonal/immunology; Bungarotoxins/pharmacology; Cell Membrane/drug effects/metabolism; Histocytochemistry; Human; Muscles/metabolism/pathology; Neuromuscular Junction/*metabolism; Prions/immunology/*metabolism; Receptors, Cholinergic/drug effects; Support, Non-U.S. Gov't; Synapses/*metabolism
AD Department of Neurology, University of Southern California School of Medicine, Los Angeles 90017-1912.
SP englisch
PO Irland