Axonal transport of the cellular prion protein is increased during axon regeneration

Abstract

The cellular prion protein, PrP

Dramatic Reduction of PrPCLevel and Glycosylation in Peripheral Nerves following PrP Knock-Out from Schwann Cells Does Not Prevent Transmissible Spongiform Encephalopathy Neuroinvasion

“…Studies have suggested that glial cells do not express PrP C , as it could not be detected following axon degeneration with Schwann cells still present (Moya et al, 2005). In contrast PrP C expression in glial cells (Moser et al, 1995) and specifically myelin of the CNS has previously been reported (Mironov et al, 2003).…”

Cellular prion protein electron microscopy: attempts/limits and clues to a synaptic trait. Implications in neurodegeneration process

“…Of particular note, after intraocular injection in chick embryos, endogenous or exogenous recombinant PrP c are reported to be internalized by retinal ganglion cells and then anterogradly conveyed within axons in the direction of the optic tectum (Butowt et al 2007). This highlights the driving force behind PrP c for functional destination towards the growth cone in developing axonal fibers (Moya et al 2005) or axonal synapses in the mature neuron (Borchelt et al 1994). PrP c can also use retrograde transport for escorting informative molecules to the cell body (Moya et al 2005).…”

“…This highlights the driving force behind PrP c for functional destination towards the growth cone in developing axonal fibers (Moya et al 2005) or axonal synapses in the mature neuron (Borchelt et al 1994). PrP c can also use retrograde transport for escorting informative molecules to the cell body (Moya et al 2005). In addition to a constitutive localization on the postsynaptic side (Moya et al 2000;Bailly et al 2004), PrP c might occupy this site by transynaptic migration after being freely released (Moya et al 2000;Liu et al 2002) or associated with exosomes (Fauré et al 2006).…”

“…This type of intraneuronal molecule movement constitutes a fundamental feature of PrP c biology (Borchelt et al 1994;Moya et al 2005). In transgenic mice expressing prion protein tagged with enhanced green fluorescent protein (EGFP), axonal transport is essential to deliver PrP-EGFP to the presynaptic site (Barmada et al 2004).…”

The Protein-disulfide Isomerase ERp57 Regulates the Steady-state Levels of the Prion Protein

“…We have recently reported a functional characterization of this transgenic line showing that overexpression of ERp57 improves recovery in peripheral nerve regeneration after mechanical injury to sciatic nerve (57), indicating that the overexpressed chaperone is functional in the nervous system. Interestingly, PrP expression is induced in models of peripheral nerve degeneration (68), and its deficiency triggers myelin defects (69). Because mice overexpressing ERp57 displayed augmented levels of PrP in the nervous system, it remains to be determined whether part of the beneficial effects of overexpressing ERp57 are due to enhancement of PrP expression.…”