Neuronal low-density lipoprotein receptor-related protein 1 binds and endocytoses prion fibrils via receptor cluster 4.

Journal article

For infectious prion protein (designated PrP(Sc)) to act as a template to convert normal cellular protein (PrP(C)) to its distinctive pathogenic conformation, the two forms of prion protein (PrP) must interact closely. The neuronal receptor that rapidly endocytoses PrP(C) is the low-density lipoprotein receptor-related protein 1 (LRP1). We show here that on sensory neurons LRP1 is also the receptor that binds and rapidly endocytoses smaller oligomeric forms of infectious prion fibrils, and re...

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Authors: Jen, A; Parkyn, C; Mootoosamy, R; Ford, M; Warley, A

• Publication status: Published
• Journal: Journal of cell science
• Volume: 123
• Issue: Pt 2
• Pages: 246-255
• Publication date: 2010-01-01
• DOI: 10.1242/jcs.058099
• EISSN: 1477-9137
• ISSN: 0021-9533
• Language: English
• Keywords: Prions; Endocytosis; Protein Structure, Secondary; Mice; PrPC Proteins; Lysosomes; Endosomes; Animals; PrPSc Proteins; Cells, Cultured; Ligands; Sensory Receptor Cells; Peptide Hydrolases; Tumor Suppressor Proteins; Receptors, LDL; Protein Binding; Mice, Inbred C57BL