Function of PrP(C) as a copper-binding protein at the synapse.
The prion protein (PrP(C)) shows cooperative copper binding of the N-terminal octarepeat (PHGGGWGO) x4. In brain homogenates, PrP(C) is found in highest concentration in synaptosomal fractions. Mice devoid of PrP(C) (Prnp0/0 mice) show synaptosomal copper concentrations diminished by 50% as compared to normal mice. PrP(C) in the synaptic cleft may serve as a copper buffer. Alternatively it may play a role in the re-uptake of copper into the presynapse or may be of structural importance for the N-terminus and thus may influence binding of PrP(C) to other proteins.
Kretzschmar
H A
HA
Institute of Neuropathology, University of Göttingen, Germany.
Tings
T
T
Madlung
A
A
Giese
A
A
Herms
J
J
D000818:Animals; D001921:Brain / Q000378:metabolism*; D002352:Carrier Proteins / Q000378:metabolism*; D003300:Copper / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D018621:PrPC Proteins / Q000235:genetics / Q000378:metabolism*; D013569:Synapses / Q000378:metabolism*
D002352:Carrier Proteins; D018621:PrPC Proteins; C023548:copper-binding protein; D003300:Copper