Prion protein is strongly immunolocalized at the postsynaptic domain of human normal neuromuscular junctions.
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.
Askanas
V
V
Department of Neurology, University of Southern California School of Medicine, Los Angeles 90017-1912.
Bilak
M
M
Engel
W K
WK
Leclerc
A
A
Tomé
F
F
D000911:Antibodies, Monoclonal / Q000276:immunology; D002038:Bungarotoxins / Q000494:pharmacology; D002462:Cell Membrane / Q000187:drug effects / Q000378:metabolism; D006651:Histocytochemistry; D006801:Humans; D009132:Muscles / Q000378:metabolism / Q000473:pathology; D009469:Neuromuscular Junction / Q000378:metabolism*; D011328:Prions / Q000276:immunology / Q000378:metabolism*; D011950:Receptors, Cholinergic / Q000187:drug effects; D013569:Synapses / Q000378:metabolism*
D000911:Antibodies, Monoclonal; D002038:Bungarotoxins; D011328:Prions; D011950:Receptors, Cholinergic