Prion protein: Difference between revisions

New page: The prion protein (PrP) is a cell surface glycoprotein. The cellular isoform (PrPC) is predominantly α-helical, but can undergo a structural conversion to a β-sheet rich conformation, te...
 
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The prion protein (PrP) is a cell surface glycoprotein. The cellular isoform (PrPC) is predominantly α-helical, but can undergo a structural conversion to a β-sheet rich conformation, termed PrPSc. 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 PrPSc
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.
Structure from  a


   's normal cellular function is debated, and "knockout" mice lacking PrP are phenotypically normal.
   's normal cellular function is debated, and "knockout" mice lacking PrP are phenotypically normal.
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{{STRUCTURE_1hjm |  PDB=1hjm  |  SCENE=  }}
{{STRUCTURE_1hjm |  PDB=1hjm  |  SCENE=  }}
==PrP structures==
1AG2 Mouse PrP 121-231 determined by NMR
1B10 Syrian hamster PrP 90-231 NMR ensemble of 25 structures
1DWY BoPrP              121-230 Average            124-227
1DWZ BoPrP              121-230 20 structures      124-227
1DX0 BoPrP                23-230 Average            124-227
1DX1 BoPrP                23-230 20 structures      124-227
1E1G HuPrP,M166V        125-228 20 structures      125-228
1E1J HuPrP,M166V        125-228 Average            125-228
1E1P HuPrP,S170N        125-228 20 structures      125-228
1E1S HuPrP,S170N        125-228 Average            125-228
1E1U HuPrP,R220K        125-228 20 structures      125-228
1E1W HuPrP,R220K        125-228 Average            125-228
1FKC HuPrP,E200K          90-231 Average            125-231
1FO7 HuPrP,E200K          90-231 30 structures      125-231
1HJM HuPrP                      Average
1HJN HuPrP                      20 structures
1H0L HuPrP,M166C,E221C  121-230 20 structures      119-230
1I4M HuPrP              119-226 (X-ray)            119-226
1QLX HuPrP                23-230 Average            125-228
1QLZ HuPrP                23-230 20 structures      125-228
1QM0 HuPrP                90-230 Average            125-228
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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Kurt Giles, Jaime Prilusky, Eran Hodis, Claudio Garutti, Michal Harel, Joel L. Sussman