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>) is predominantly α-helical, but 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 from  a  
Structure from  a  


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==PrP structures==
==Selected PrP structures==
1AG2 Mouse PrP 121-231 determined by NMR
=Human PrP=
1B10 Syrian hamster PrP 90-231 NMR ensemble of 25 structures
* 1QLX HuPrP                23-230 Average            125-228
1DWY BoPrP              121-230 Average            124-227
* 1QM0 HuPrP               90-230 Average            125-228
1DWZ BoPrP              121-230 20 structures      124-227
* 1QM2 HuPrP              121-230 Average            125-228
1DX0 BoPrP               23-230 Average            124-227
* 1I4M HuPrP               119-226 (X-ray)            119-226
1DX1 BoPrP                23-230 20 structures      124-227
* 1E1J HuPrP,M166V        125-228 Average            125-228
1E1G HuPrP,M166V        125-228 20 structures      125-228
* 1E1S HuPrP,S170N        125-228 Average            125-228
1E1J HuPrP,M166V        125-228 Average            125-228
* 1E1W HuPrP,R220K        125-228 Average            125-228
1E1P HuPrP,S170N        125-228 20 structures      125-228
* 1FKC HuPrP,E200K          90-231 Average            125-231
1E1S HuPrP,S170N        125-228 Average            125-228
* 1H0L HuPrP,M166C,E221C  121-230 20 structures      119-230
1E1U HuPrP,R220K        125-228 20 structures      125-228
 
1E1W HuPrP,R220K        125-228 Average            125-228
=Other PrPs=
1FKC HuPrP,E200K          90-231 Average            125-231
=Rodent PrP=
1FO7 HuPrP,E200K          90-231 30 structures      125-231
* XXXX Mouse PrP determined by NMR
1HJM HuPrP                      Average
 
1HJN HuPrP                      20 structures
* 1B10 Syrian hamster PrP 90-231 NMR ensemble of 25 structures
1H0L HuPrP,M166C,E221C  121-230 20 structures      119-230
1DWY Cow PrP               121-230 Average            124-227
1I4M HuPrP              119-226 (X-ray)            119-226
1UW3 Sheep PrP
1QLX HuPrP                23-230 Average            125-228
* XXXX Frog PrP
1QLZ HuPrP                23-230 20 structures      125-228
* Chicken PrP
1QM0 HuPrP                90-230 Average            125-228
* Turtle PrP
1QM1 HuPrP                90-230 20 structures     125-228
1QM2 HuPrP               121-230 Average            125-228
1QM3 HuPrP              121-230 20 structures      125-228
1UW3 OvPrP
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