Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia

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...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Anna Hofmann, Arne Wrede, Wiebke M. Jürgens-Wemheuer, Walter J. Schulz-Schaeffer
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Transmissible spongiform encephalopathies Protein aggregation disease Celiac ganglion Sympathetic trunk ganglia PET-blot Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/d293f9a2efb24f738a2a263a001ade82
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:d293f9a2efb24f738a2a263a001ade82
record_format	dspace
spelling	oai:doaj.org-article:d293f9a2efb24f738a2a263a001ade822021-11-28T12:09:11ZPrion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia10.1186/s40478-021-01286-42051-5960https://doaj.org/article/d293f9a2efb24f738a2a263a001ade822021-11-01T00:00:00Zhttps://doi.org/10.1186/s40478-021-01286-4https://doaj.org/toc/2051-5960Abstract 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.Anna HofmannArne WredeWiebke M. Jürgens-WemheuerWalter J. Schulz-SchaefferBMCarticleTransmissible spongiform encephalopathiesProtein aggregation diseaseCeliac ganglionSympathetic trunk gangliaPET-blotNeurology. Diseases of the nervous systemRC346-429ENActa Neuropathologica Communications, Vol 9, Iss 1, Pp 1-10 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Transmissible spongiform encephalopathies Protein aggregation disease Celiac ganglion Sympathetic trunk ganglia PET-blot Neurology. Diseases of the nervous system RC346-429
spellingShingle	Transmissible spongiform encephalopathies Protein aggregation disease Celiac ganglion Sympathetic trunk ganglia PET-blot Neurology. Diseases of the nervous system RC346-429 Anna Hofmann Arne Wrede Wiebke M. Jürgens-Wemheuer Walter J. Schulz-Schaeffer Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
description	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.
format	article
author	Anna Hofmann Arne Wrede Wiebke M. Jürgens-Wemheuer Walter J. Schulz-Schaeffer
author_facet	Anna Hofmann Arne Wrede Wiebke M. Jürgens-Wemheuer Walter J. Schulz-Schaeffer
author_sort	Anna Hofmann
title	Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
title_short	Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
title_full	Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
title_fullStr	Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
title_full_unstemmed	Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
title_sort	prion type 2 selection in sporadic creutzfeldt–jakob disease affecting peripheral ganglia
publisher	BMC
publishDate	2021
url	https://doaj.org/article/d293f9a2efb24f738a2a263a001ade82
work_keys_str_mv	AT annahofmann priontype2selectioninsporadiccreutzfeldtjakobdiseaseaffectingperipheralganglia AT arnewrede priontype2selectioninsporadiccreutzfeldtjakobdiseaseaffectingperipheralganglia AT wiebkemjurgenswemheuer priontype2selectioninsporadiccreutzfeldtjakobdiseaseaffectingperipheralganglia AT walterjschulzschaeffer priontype2selectioninsporadiccreutzfeldtjakobdiseaseaffectingperipheralganglia
_version_	1718408168401272832

Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia

Ejemplares similares