Prion protein: Difference between revisions
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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-terminus 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. | ||
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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=Genetic prion diseases= | =Genetic prion diseases= | ||
A number of mutations in PrP have been identified which correlate with a high incidence of prion disease. To date, structural studies of all mutant PrP<sup>C</sup> have extremely similar structures to wild type PrP<sup>C</sup>, suggesting a kinetic basis for the difference in converting to PrP<sup>Sc</sup>. | A number of mutations in PrP have been identified which correlate with a high incidence of prion disease. The structure of HuPrP,E200K was determined nd shown to be To date, structural studies of all mutant PrP<sup>C</sup> have extremely similar structures to wild type PrP<sup>C</sup>, suggesting a kinetic basis for the difference in converting to PrP<sup>Sc</sup>. | ||
=Prion strains= | =Prion strains= | ||
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* [[1QM2]] HuPrP residues 121-230 | * [[1QM2]] HuPrP residues 121-230 | ||
* [[1I4M]] HuPrP residues 119-226 (X-ray) | * [[1I4M]] HuPrP residues 119-226 (X-ray) | ||
* [[1FKC]] HuPrP,E200K residues 90-231 (genetic prion disease) | * [[1FKC]] HuPrP,E200K residues 90-231 (genetic prion disease) | ||
* [[1H0L]] HuPrP residues 121-230, with an additional disulphide bond analogous to the homolog Doppel | * [[1H0L]] HuPrP residues 121-230, with an additional disulphide bond analogous to the homolog Doppel | ||
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=References= | =References= | ||
{{Reflist}} <references/> | |||
1. | |||