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Methionine 129 variant of human prion protein oligomerizes more rapidly than the valine 129 variant: implications for disease susceptibility to Creutzfeldt-Jakob disease.

Abstract:

The human PrP gene (PRNP) has two common alleles that encode either methionine or valine at codon 129. This polymorphism modulates disease susceptibility and phenotype of human transmissible spongiform encyphalopathies, but the molecular mechanism by which these effects are mediated remains unclear. Here, we compared the misfolding pathway that leads to the formation of beta-sheet-rich oligomeric isoforms of the methionine 129 variant of PrP to that of the valine 129 variant. We provide evide...

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Publication status:
Published

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Publisher copy:
10.1074/jbc.m401754200

Authors


Journal:
The Journal of biological chemistry
Volume:
279
Issue:
30
Pages:
31390-31397
Publication date:
2004-07-05
DOI:
EISSN:
1083-351X
ISSN:
0021-9258
URN:
uuid:d3b3a6ca-7c6e-406b-9e4f-778abbd488b7
Source identifiers:
22358
Local pid:
pubs:22358

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