Prion protein: Difference between revisions
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=Prion diseases= | =Prion diseases= | ||
The naturally ocuring prion diseases include Creutzfeldt Jakob disease (CJD) in people, bovine spongiform encephalopathy (BSE) commonly known as "mad cow" disease,scpie in sheep and goats, and chronic wasting disease in cervids | The naturally ocuring prion diseases include Creutzfeldt Jakob disease (CJD) in people, bovine spongiform encephalopathy (BSE) commonly known as "mad cow" disease,scpie in sheep and goats, and chronic wasting disease in cervids are characterterized by aggregates of PrP<sup>Sc</sup>. | ||
The spontaneous, genetic and infectious etiologies of prion diseases can be explained by a simple protein-based model in which PrP<sup>C</sup> is converted into PrP<sup>Sc</sup>, which then initiates autocatalytic refolding of PrP<sup>C</sup> in a template-dependent manner. | The spontaneous, genetic and infectious etiologies of prion diseases can be explained by a simple protein-based model in which PrP<sup>C</sup> is converted into PrP<sup>Sc</sup>, which then initiates autocatalytic refolding of PrP<sup>C</sup> in a template-dependent manner. | ||
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=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-terminal region has little impact on the structure of the C-terminal domain <ref> | 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>Zahn, R. ''et al.'' (2000) NMR solution structure of the human prion protein ''Proc. Natl. Acad. Sci. USA'' '''97''', 145-150 </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. | ||
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* [[1u5l]] Turtle PrP residues 121-226 | * [[1u5l]] Turtle PrP residues 121-226 | ||
=References= | =References= | ||
<references/> | <references/> | ||
