000144786 001__ 144786 000144786 005__ 20250718162053.0 000144786 0247_ $$2URN$$aurn:nbn:de:hbz:5n-47847 000144786 037__ $$aDZNE-2020-00229 000144786 041__ $$aEnglish 000144786 1001_ $$0P:(DE-2719)2810477$$aBreid, Sara$$b0$$eFirst author$$udzne 000144786 245__ $$aTransmission of pathogenic alpha-synuclein to mice$$f - 2017-06-12 000144786 260__ $$aBonn$$c2017 000144786 300__ $$a111 pages 000144786 3367_ $$2DataCite$$aOutput Types/Dissertation 000144786 3367_ $$2ORCID$$aDISSERTATION 000144786 3367_ $$2BibTeX$$aPHDTHESIS 000144786 3367_ $$02$$2EndNote$$aThesis 000144786 3367_ $$0PUB:(DE-HGF)11$$2PUB:(DE-HGF)$$aDissertation / PhD Thesis$$bphd$$mphd$$s1752848396_2393 000144786 3367_ $$2DRIVER$$adoctoralThesis 000144786 502__ $$aDissertation, Rheinische Friedrich-Wilhelms-Universität Bonn, 2017$$bDissertation$$cRheinische Friedrich-Wilhelms-Universität Bonn$$d2017 000144786 520__ $$aα-Synuclein is a soluble, cellular protein that in a number of neurodegenerative diseases, including Parkinson's disease, multiple system atrophy, and Lewy body dementia aggregates into pathological protein deposits. Principles how misfolded and aggregated α-synuclein is transmitted within the central nervous system (CNS) causing neurologic disease were found to be similar to those of prions. Misfolded α-synuclein can be transmitted between cells and act as a seed, recruiting native, unfolded α-synuclein to form insoluble aggregates. The mechanisms and the routes through which pathogenic proteins enter the CNS causing progressive disease are still not completely understood. The work in this thesis confirms previous findings indicating that α-synuclein fibrils intracerebrally injected into wild-type mice for α-synuclein can induce neuropathology in interconnected brain regions as similarly observed in sporadic Parkinson's disease. In contrast, α-synuclein fibrils injected into the tongue muscle of wild-type mice for α-synuclein did not neuroinvade the CNS causing α-synuclein pathology. Moreover, the present study is the first to show, that α-synuclein fibrils peripherally injected into the tongue and the peritoneum of mice overexpressing human α-synuclein, can neuroinvade the CNS, cause widespread α-synuclein pathology and induce neurologic symptoms. The induction of neuropathology was accompanied by neuroinflammation as monitored by astrocytic gliosis and microgliosis. In addition, the study presented here indicates that exposure of mice overexpressing human α-synuclein with pathogenic α-synuclein aerosols was not sufficient for α-synuclein prionoids to enter the brain via the olfactory epithelium and induce neuropathology. In summary, these findings corroborate the prionoid character of misfolded α-synuclein using similar routes like prions to neuroinvade brain and spinal cord and induce neurologic disease. 000144786 536__ $$0G:(DE-HGF)POF3-342$$a342 - Disease Mechanisms and Model Systems (POF3-342)$$cPOF3-342$$fPOF III$$x0 000144786 8564_ $$uhttps://hdl.handle.net/20.500.11811/7209 000144786 8564_ $$uhttps://pub.dzne.de/record/144786/files/DZNE-2020-00229_Restricted.pdf 000144786 8564_ $$uhttps://pub.dzne.de/record/144786/files/DZNE-2020-00229_Restricted.pdf?subformat=pdfa$$xpdfa 000144786 909CO $$ooai:pub.dzne.de:144786$$pVDB 000144786 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810477$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b0$$kDZNE 000144786 9131_ $$0G:(DE-HGF)POF3-342$$1G:(DE-HGF)POF3-340$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lErkrankungen des Nervensystems$$vDisease Mechanisms and Model Systems$$x0 000144786 9141_ $$y2017 000144786 920__ $$lyes 000144786 9201_ $$0I:(DE-2719)1013022$$kAG Tamgüney$$lPrion and prion-like diseases$$x0 000144786 980__ $$aphd 000144786 980__ $$aVDB 000144786 980__ $$aI:(DE-2719)1013022 000144786 980__ $$aUNRESTRICTED