Please use this identifier to cite or link to this item:
doi:10.22028/D291-35778
Title: | Prion type 2 selection in sporadic Creutzfeldt-Jakob disease affecting peripheral ganglia |
Author(s): | Hofmann, Anna Wrede, Arne Jürgens-Wemheuer, Wiebke M. Schulz-Schaeffer, Walter J. |
Language: | English |
Title: | Acta Neuropathologica Communications |
Volume: | 9 |
Issue: | 1 |
Publisher/Platform: | BMC |
Year of Publication: | 2021 |
Free key words: | Transmissible spongiform encephalopathies Protein aggregation disease Celiac ganglion Sympathetic trunk ganglia PET-blot |
DDC notations: | 610 Medicine and health |
Publikation type: | Journal Article |
Abstract: | In sporadic Creutzfeldt–Jakob disease (sCJD), the pathological changes appear to be restricted to the central nervous system. Only involvement of the trigeminal ganglion is widely accepted. The present study systematically examined the involvement of peripheral ganglia in sCJD utilizing the currently most sensitive technique for detecting prions in tissue morphologically. The trigeminal, nodose, stellate, and celiac ganglia, as well as ganglia of the cervical, thoracic and lumbar sympathetic trunk of 40 patients were analyzed with the paraffin-embedded tissue (PET)-blot method. Apart from the trigeminal ganglion, which contained protein aggregates in five of 19 prion type 1 patients, evidence of prion protein aggregation was only found in patients associated with type 2 prions. With the PET-blot, aggregates of prion protein type 2 were found in all trigeminal (17/17), in some nodose (5 of 7) and thoracic (3 of 6) ganglia, as well as in a few celiac (4 of 19) and lumbar (1 of 5) ganglia of sCJD patients. Whereas aggregates of both prion types may spread to dorsal root ganglia, more CNS-distant ganglia seem to be only involved in patients accumulating prion type 2. Whether the prion type association is due to selection by prion type-dependent replication, or due to a prion type-dependent property of axonal spread remains to be resolved in further studies. |
DOI of the first publication: | 10.1186/s40478-021-01286-4 |
Link to this record: | urn:nbn:de:bsz:291--ds-357782 hdl:20.500.11880/32622 http://dx.doi.org/10.22028/D291-35778 |
ISSN: | 2051-5960 |
Date of registration: | 17-Mar-2022 |
Description of the related object: | Supplementary Information |
Related object: | https://ndownloader.figstatic.com/files/31577174 |
Faculty: | M - Medizinische Fakultät |
Department: | M - Neuropathologie |
Professorship: | M - Prof. Dr. Walter Schulz-Schaeffer |
Collections: | SciDok - Der Wissenschaftsserver der Universität des Saarlandes |
Files for this record:
File | Description | Size | Format | |
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s40478-021-01286-4.pdf | 1,88 MB | Adobe PDF | View/Open |
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