1qlz
HUMAN PRION PROTEIN
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OverviewOverview
The NMR structures of the recombinant human prion protein, hPrP(23-230), and two C-terminal fragments, hPrP(90-230) and hPrP(121-230), include a, globular domain extending from residues 125-228, for which a detailed, structure was obtained, and an N-terminal flexibly disordered "tail." The, globular domain contains three alpha-helices comprising the residues, 144-154, 173-194, and 200-228 and a short anti-parallel beta-sheet, comprising the residues 128-131 and 161-164. Within the globular domain, three polypeptide segments show increased structural disorder: i.e., a, loop of residues 167-171, the residues 187-194 at the end of helix 2, and, the residues 219-228 in the C-terminal part of helix 3. The local, conformational state of the polypeptide segments 187-193 in helix 2 and, 219-226 in helix 3 is measurably influenced by the length of the, N-terminal tail, with the helical states being most highly populated in, hPrP(23-230). When compared with the previously reported structures of the, murine and Syrian hamster prion proteins, the length of helix 3 coincides, more closely with that in the Syrian hamster protein whereas the, disordered loop 167-171 is shared with murine PrP. These species, variations of local structure are in a surface area of the cellular form, of PrP that has previously been implicated in intermolecular interactions, related both to the species barrier for infectious transmission of prion, disease and to immune reactions.
DiseaseDisease
Known diseases associated with this structure: Creutzfeldt-Jakob disease OMIM:[176640], Gerstmann-Straussler disease OMIM:[176640], Huntington disease-like 1 OMIM:[176640], Insomnia, fatal familial OMIM:[176640], Prion disease with protracted course OMIM:[176640], Retinitis pigmentosa-11 OMIM:[606419]
About this StructureAbout this Structure
1QLZ is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
NMR solution structure of the human prion protein., Zahn R, Liu A, Luhrs T, Riek R, von Schroetter C, Lopez Garcia F, Billeter M, Calzolai L, Wider G, Wuthrich K, Proc Natl Acad Sci U S A. 2000 Jan 4;97(1):145-50. PMID:10618385
Page seeded by OCA on Fri Feb 15 16:45:06 2008