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| [[Image:1fkc.gif|left|200px]] | | {{Seed}} |
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| {{STRUCTURE_1fkc| PDB=1fkc | SCENE= }} | | {{STRUCTURE_1fkc| PDB=1fkc | SCENE= }} |
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| '''HUMAN PRION PROTEIN (MUTANT E200K) FRAGMENT 90-231'''
| | ===HUMAN PRION PROTEIN (MUTANT E200K) FRAGMENT 90-231=== |
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| ==Overview==
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| Prion propagation in transmissible spongiform encephalopathies involves the conversion of cellular prion protein, PrP(C), into a pathogenic conformer, PrP(Sc). Hereditary forms of the disease are linked to specific mutations in the gene coding for the prion protein. To gain insight into the molecular basis of these disorders, the solution structure of the familial Creutzfeldt-Jakob disease-related E200K variant of human prion protein was determined by multi-dimensional nuclear magnetic resonance spectroscopy. Remarkably, apart from minor differences in flexible regions, the backbone tertiary structure of the E200K variant is nearly identical to that reported for the wild-type human prion protein. The only major consequence of the mutation is the perturbation of surface electrostatic potential. The present structural data strongly suggest that protein surface defects leading to abnormalities in the interaction of prion protein with auxiliary proteins/chaperones or cellular membranes should be considered key determinants of a spontaneous PrP(C) --> PrP(Sc) conversion in the E200K form of hereditary prion disease.
| | The line below this paragraph, {{ABSTRACT_PUBMED_10954699}}, adds the Publication Abstract to the page |
| | (as it appears on PubMed at http://www.pubmed.gov), where 10954699 is the PubMed ID number. |
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| | {{ABSTRACT_PUBMED_10954699}} |
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| ==About this Structure== | | ==About this Structure== |
| 1FKC is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1FKC OCA]. | | 1FKC is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1FKC OCA]. |
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| ==Reference== | | ==Reference== |
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| [[Category: Prion]] | | [[Category: Prion]] |
| [[Category: Three helix]] | | [[Category: Three helix]] |
| ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 16:25:35 2008'' | | |
| | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 03:23:43 2008'' |