Strain-Dependent Prion Infection in Mice Expressing Prion Protein with Deletion of Central Residues 91–106
| العنوان: | Strain-Dependent Prion Infection in Mice Expressing Prion Protein with Deletion of Central Residues 91–106 |
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| المؤلفون: | Agriani Dini Pasiana, Junji Chida, Mariya Okazaki, Ryuichiro Atarashi, Hitomi Watanabe, Suehiro Sakaguchi, Gen Kondoh, Hideyuki Hara, Keiji Uchiyama, Hironori Miyata, Morikazu Imamura, Yoshitaka Yamaguchi |
| المصدر: | International Journal of Molecular Sciences Volume 21 Issue 19 International Journal of Molecular Sciences, Vol 21, Iss 7260, p 7260 (2020) |
| بيانات النشر: | Multidisciplinary Digital Publishing Institute, 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | 0301 basic medicine, PrPSc Proteins, animal diseases, Gene Expression, law.invention, lcsh:Chemistry, Pathogenesis, chemistry.chemical_compound, Mice, law, Cloning, Molecular, prions, protein misfolding, lcsh:QH301-705.5, Spectroscopy, Sequence Deletion, Chemistry, Neurodegeneration, neurodegeneration, Brain, General Medicine, Recombinant Proteins, Computer Science Applications, Encephalopathy, Bovine Spongiform, Digitonin, Recombinant DNA, Protein folding, Disease Susceptibility, Baculoviridae, Gene isoform, Genetically modified mouse, Bovine spongiform encephalopathy, Mice, Transgenic, transgenic mice, Catalysis, Article, Inorganic Chemistry, 03 medical and health sciences, Species Specificity, medicine, Animals, PrPC Proteins, Physical and Theoretical Chemistry, Proteostasis Deficiencies, Molecular Biology, Injections, Intraventricular, 030102 biochemistry & molecular biology, Base Sequence, Organic Chemistry, medicine.disease, Molecular biology, nervous system diseases, 030104 developmental biology, lcsh:Biology (General), lcsh:QD1-999, nervous system, prion protein, Cattle, Scrapie |
| الوصف: | Conformational conversion of the cellular prion protein, PrPC, into the abnormally folded isoform, PrPSc, is a key pathogenic event in prion diseases. However, the exact conversion mechanism remains largely unknown. Transgenic mice expressing PrP with a deletion of the central residues 91&ndash 106 were generated in the absence of endogenous PrPC, designated Tg(PrP∆91&ndash 106)/Prnp0/0 mice and intracerebrally inoculated with various prions. Tg(PrP∆91&ndash 106)/Prnp0/0 mice were resistant to RML, 22L and FK-1 prions, neither producing PrPSc∆91&ndash 106 or prions in the brain nor developing disease after inoculation. However, they remained marginally susceptible to bovine spongiform encephalopathy (BSE) prions, developing disease after elongated incubation times and accumulating PrPSc∆91&ndash 106 and prions in the brain after inoculation with BSE prions. Recombinant PrP∆91-104 converted into PrPSc∆91&ndash 104 after incubation with BSE-PrPSc-prions but not with RML- and 22L&ndash PrPSc-prions, in a protein misfolding cyclic amplification assay. However, digitonin and heparin stimulated the conversion of PrP∆91&ndash 104 into PrPSc∆91&ndash 104 even after incubation with RML- and 22L-PrPSc-prions. These results suggest that residues 91&ndash 106 or 91&ndash 104 of PrPC are crucially involved in prion pathogenesis in a strain-dependent manner and may play a similar role to digitonin and heparin in the conversion of PrPC into PrPSc. |
| وصف الملف: | application/pdf |
| اللغة: | English |
| تدمد: | 1422-0067 |
| DOI: | 10.3390/ijms21197260 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6c85d2d4e530c113b28fa34ec47df45eTest |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....6c85d2d4e530c113b28fa34ec47df45e |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14220067 |
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| DOI: | 10.3390/ijms21197260 |