Prion protein: Difference between revisions

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Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Kurt Giles, Jaime Prilusky, Eran Hodis, Claudio Garutti, Michal Harel, Joel L. Sussman

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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>=
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 =Selected PrP structures=
+All structures determined by NMR unless otherwise specified
 ==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
-* 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==
 * XXXX Mouse PrP determined by NMR
 * [[1B10]] Syrian hamster PrP 90-231 NMR ensemble of 25 structures
 * [[1DWY]] Cow PrP               121-230 Average            124-227
-* [[1UW3]] Sheep PrP
+* [[1UW3]] Sheep PrP (X ray)
 * XXXX Frog PrP
 * XXXX Chicken PrP