Prion protein: Difference between revisions
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The prion protein (PrP) is a cell surface glycoprotein. The cellular isoform (PrP<sup>C</sup>) has two dominas: an dN-terminal region that is natively unstructured, and a C-terminal region from residues ~120-230, which is predominantly α-helical. PrP<sup>C</sup> can undergo a structural conversion to a β-sheet rich conformation, 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. | The prion protein (PrP) is a cell surface glycoprotein. The cellular isoform (PrP<sup>C</sup>) has two dominas: an dN-terminal region that is natively unstructured, and a C-terminal region from residues ~120-230, which is predominantly α-helical. PrP<sup>C</sup> can undergo a structural conversion to a β-sheet rich conformation, 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_1hjm | PDB=1hjm | SCENE= }} | {{STRUCTURE_1hjm | PDB=1hjm | SCENE= }} | ||
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The X-ray structure of sheep PrP was dimeric... | The X-ray structure of sheep PrP was dimeric... | ||
=Models of PrP<sup>Sc</sup> structure= | |||
There are a number of technical obstacles in determining the molecular structure of PrP(sup)Sc</sup> | There are a number of technical obstacles in determining the molecular structure of PrP(sup)Sc</sup> | ||
=Selected PrP structures= | |||
=Human PrP= | ==Human PrP== | ||
* [[1QLX]] HuPrP 23-230 Average 125-228 | * [[1QLX]] HuPrP 23-230 Average 125-228 | ||
* [[1QM0]] HuPrP 90-230 Average 125-228 | * [[1QM0]] HuPrP 90-230 Average 125-228 | ||
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* 1H0L HuPrP,M166C,E221C 121-230 20 structures 119-230 | * 1H0L HuPrP,M166C,E221C 121-230 20 structures 119-230 | ||
=Other PrPs= | ==Other PrPs== | ||
* XXXX Mouse PrP determined by NMR | * XXXX Mouse PrP determined by NMR | ||
* 1B10 Syrian hamster PrP 90-231 NMR ensemble of 25 structures | * [[1B10]] Syrian hamster PrP 90-231 NMR ensemble of 25 structures | ||
1DWY Cow PrP 121-230 Average 124-227 | * [[1DWY]] Cow PrP 121-230 Average 124-227 | ||
1UW3 Sheep PrP | * [[1UW3]] Sheep PrP | ||
* XXXX Frog PrP | * XXXX Frog PrP | ||
* XXXX Chicken PrP | * XXXX Chicken PrP | ||
* XXXX Turtle PrP | * XXXX Turtle PrP | ||
=References= | |||