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 a natively unstructured N-terminal region, and a predominantly α-helical C-terminal region from residues ~120-230. 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 of PrP<sup>C</sup>=
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=Selected PrP structures=
=Selected PrP structures=
All structures determined by NMR unless otherwise specified
==Human PrP==
==Human PrP==
* [[1QLX]] HuPrP                23-230 Average            125-228
* [[1QLX]] HuPrP                23-230  
* [[1QM0]] HuPrP                90-230 Average            125-228
* [[1QM0]] HuPrP                90-230  
* [[1QM2]] HuPrP              121-230 Average            125-228
* [[1QM2]] HuPrP              121-230  
* [[1I4M]] HuPrP              119-226 (X-ray)           119-226
* [[1I4M]] HuPrP              119-226 (X-ray)  
* [[1E1J]] HuPrP,M166V        125-228 Average            125-228
* [[1E1J]] HuPrP,M166V        125-228 Average            125-228
* 1E1S HuPrP,S170N        125-228 Average            125-228
* [[1E1S]] HuPrP,S170N        125-228 Average            125-228
* 1E1W HuPrP,R220K        125-228 Average            125-228
* [[1E1W]] HuPrP,R220K        125-228 Average            125-228
* 1FKC HuPrP,E200K         90-231 Average            125-231
* [[1FKC]] HuPrP,E200K residues  90-231 (genetic prion disease)
* 1H0L HuPrP,M166C,E221C  121-230 20 structures      119-230
* [[1H0L]] HuPrP residues 121-230, with an additional disulphide bond analogous to the homolog Doppel


==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 (X ray)
* XXXX Frog PrP
* XXXX Frog PrP
* XXXX Chicken PrP
* XXXX Chicken PrP
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