Prion protein: Difference between revisions
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The prion protein (PrP) is a cell surface glycoprotein | The prion protein (PrP) is a cell surface glycoprotein, which can exist in two alternatively folded confirmations: the cellular isoform (PrP<sup>C</sup>) can undergo a structural conversion to a 'scrapie' or disease associated isoform termed PrP<sup>Sc</sup>. Prion diseases such as Creutzfeldt Jakob disease (CJD) in people, and bovine spongiform encephalopathy (BSE) commonly known as "mad cow" disease, are characterterized by aggregates of PrP<sup>Sc</sup>, which arise from autocatalytic refolding of PrP<sup>C</sup> in a template-dependent manner. | ||
=Structure of PrP<sup>C</sup>= | =Structure of PrP<sup>C</sup>= | ||
{{STRUCTURE_1hjm | PDB=1hjm | SCENE= }} | {{STRUCTURE_1hjm | PDB=1hjm | SCENE= }} | ||
PrP<sup>C</sup> has a natively unstructured N-terminal region, and a predominantly α-helical C-terminal region from residues ~120-230, with a single disulfide bond. The presence of the N- | PrP<sup>C</sup> has a natively unstructured N-terminal region, and a predominantly α-helical C-terminal region from residues ~120-230, with a single disulfide bond. The presence of the N-terminal region has little impact on the structure of the C-terminal domain <ref>1</ref>. | ||
The structure is highly conserved amongst mammals, and only differs slightly in birds, reptiles and amphibians. | The structure is highly conserved amongst mammals, and only differs slightly in birds, reptiles and amphibians. | ||
The vast majority of structures have been determined by | |||
Although having a similar overall fold, the X-ray structure of sheep PrP was dimeric | |||
=Models of PrP<sup>Sc</sup> structure= | =Models of PrP<sup>Sc</sup> structure= | ||
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{{Reflist}} | {{Reflist}} | ||
Zahn, R. ''et al.'' (2000) NMR solution structure of the human prion protein ''Proc. Natl. Acad. Sci. USA'' '''97''', 145-150 | Zahn, R. ''et al.'' (2000) NMR solution structure of the human prion protein ''Proc. Natl. Acad. Sci. USA'' '''97''', 145-150 | ||
<references/> | <references/> | ||