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
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
ISSN: 2051-5960
Date of registration: 17-Mar-2022
Description of the related object: Supplementary Information
Related object:
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 SizeFormat 
s40478-021-01286-4.pdf1,88 MBAdobe PDFView/Open

This item is licensed under a Creative Commons License Creative Commons