Prion type 2 selection in sporadic Creutzfeldt-Jakob disease affecting peripheral ganglia
| Autor: | Wiebke M. Jürgens-Wemheuer, Anna Hofmann, Walter J. Schulz-Schaeffer, Arne Wrede |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
medicine.medical_specialty
Pathology Neurology Prions animal diseases Central nervous system pathology [Peripheral Nerves] Protein aggregation Biology Protein aggregation disease Creutzfeldt-Jakob Syndrome metabolism [Peripheral Nerves] Pathology and Forensic Medicine Prion Diseases Cellular and Molecular Neuroscience Trigeminal ganglion pathology [Ganglia Spinal] Ganglia Spinal metabolism [Creutzfeldt-Jakob Syndrome] medicine Humans ddc:610 Peripheral Nerves PET-blot RC346-429 Pathological Sympathetic trunk ganglia metabolism [Trigeminal Ganglion] pathology [Creutzfeldt-Jakob Syndrome] Ganglia Sympathetic Celiac ganglion Research Sporadic Creutzfeldt-Jakob disease metabolism [Ganglia Sympathetic] Peripheral nervous system diseases pathology [Trigeminal Ganglion] medicine.anatomical_structure Trigeminal Ganglion pathology [Prion Diseases] metabolism [Prions] Transmissible spongiform encephalopathies pathology [Ganglia Sympathetic] Neurology. Diseases of the nervous system Neurology (clinical) metabolism [Prion Diseases] metabolism [Ganglia Spinal] |
| Zdroj: | Acta Neuropathologica Communications Acta Neuropathologica Communications, Vol 9, Iss 1, Pp 1-10 (2021) Acta Neuropathologica Communications 9(1), 187 (2021). doi:10.1186/s40478-021-01286-4 |
| DOI: | 10.22028/d291-35778 |
| Popis: | 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. Supplementary Information The online version contains supplementary material available at 10.1186/s40478-021-01286-4. |
| Databáze: | OpenAIRE |
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