Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein Doppel
| العنوان: | Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein Doppel |
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| المؤلفون: | Robert Strome, George A. Carlson, David Westaway, Patrick Tremblay, Gregory L. Silverman, Richard C. Moore, Fred E. Cohen, Paul M. Harrison, Stanley B. Prusiner, Leroy Hood, Stephen H. Pasternak, A. Karunaratne, Kai Wang, Inyoul Lee, M A Chishti, D. W. Melton, Yan Liang, Cornelia Heinrich, Peter Mastrangelo, S. Katamine, Arian Fa Smit |
| المصدر: | ResearcherID Stephen H. Pasternak |
| مصطلحات موضوعية: | Central Nervous System, Male, Glycosylation, Prions, animal diseases, Transgene, Amino Acid Motifs, Molecular Sequence Data, Mice, Transgenic, Biology, GPI-Linked Proteins, PRNP, Cell Line, Trans-Splicing, Mice, Purkinje Cells, Downregulation and upregulation, Structural Biology, medicine, Animals, Amino Acid Sequence, RNA, Messenger, Cloning, Molecular, Molecular Biology, Gene, Peptide sequence, Messenger RNA, Mice, Inbred BALB C, Base Sequence, Neurodegeneration, medicine.disease, Embryo, Mammalian, Molecular biology, nervous system diseases, Up-Regulation, RNA splicing, Ataxia, Sequence Alignment, Gene Deletion |
| الوصف: | The novel locus Prnd is 16 kb downstream of the mouse prion protein (PrP) gene Prnp and encodes a 179 residue PrP-like protein designated doppel (Dpl). Prnd generates major transcripts of 1.7 and 2.7 kb as well as some unusual chimeric transcripts generated by intergenic splicing with Prnp. Like PrP, Dpl mRNA is expressed during embryogenesis but, in contrast to PrP, it is expressed minimally in the CNS. Unexpectedly, Dpl is upregulated in the CNS of two PrP-deficient (Prnp(0/0)) lines of mice, both of which develop late-onset ataxia, suggesting that Dpl may provoke neurodegeneration. Dpl is the first PrP-like protein to be described in mammals, and since Dpl seems to cause neurodegeneration similar to PrP, the linked expression of the Prnp and Prnd genes may play a previously unrecognized role in the pathogenesis of prion diseases or other illnesses. |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2ce2547c1d1b9f2a7b5fff3ba8defb48 http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=ORCID&SrcApp=OrcidOrg&DestLinkType=FullRecord&DestApp=WOS_CPL&KeyUT=WOS:000083134500005&KeyUID=WOS:000083134500005 |
| Rights: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....2ce2547c1d1b9f2a7b5fff3ba8defb48 |
| قاعدة البيانات: | OpenAIRE |
| الوصف غير متاح. |