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Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay
by
Matěj, Radoslav
, Moško, Tibor
, Brůžová, Magdalena
, Baranová, Soňa
, Kim, Chae
, Haldiman, Tracy
, Safar, Jiri G.
, Holada, Karel
in
631/378
/ 631/378/1689
/ Aggregates
/ Autopsies
/ Biological Assay
/ Biopsy
/ Cerebrospinal fluid
/ Creutzfeldt-Jakob disease
/ Creutzfeldt-Jakob Syndrome - cerebrospinal fluid
/ Creutzfeldt-Jakob Syndrome - diagnosis
/ Dementia
/ Hospitals
/ Humanities and Social Sciences
/ Humans
/ Lymphoma
/ Medicine
/ multidisciplinary
/ Mutation
/ Neuropathology
/ Pathology
/ Patients
/ Prion Diseases - diagnosis
/ Prion protein
/ Prion Proteins
/ Prions
/ Prions - metabolism
/ Protein seeding
/ Proteins
/ Science
/ Science (multidisciplinary)
/ Skin
/ Skin - metabolism
2024
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Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay
by
Matěj, Radoslav
, Moško, Tibor
, Brůžová, Magdalena
, Baranová, Soňa
, Kim, Chae
, Haldiman, Tracy
, Safar, Jiri G.
, Holada, Karel
in
631/378
/ 631/378/1689
/ Aggregates
/ Autopsies
/ Biological Assay
/ Biopsy
/ Cerebrospinal fluid
/ Creutzfeldt-Jakob disease
/ Creutzfeldt-Jakob Syndrome - cerebrospinal fluid
/ Creutzfeldt-Jakob Syndrome - diagnosis
/ Dementia
/ Hospitals
/ Humanities and Social Sciences
/ Humans
/ Lymphoma
/ Medicine
/ multidisciplinary
/ Mutation
/ Neuropathology
/ Pathology
/ Patients
/ Prion Diseases - diagnosis
/ Prion protein
/ Prion Proteins
/ Prions
/ Prions - metabolism
/ Protein seeding
/ Proteins
/ Science
/ Science (multidisciplinary)
/ Skin
/ Skin - metabolism
2024
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Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay
by
Matěj, Radoslav
, Moško, Tibor
, Brůžová, Magdalena
, Baranová, Soňa
, Kim, Chae
, Haldiman, Tracy
, Safar, Jiri G.
, Holada, Karel
in
631/378
/ 631/378/1689
/ Aggregates
/ Autopsies
/ Biological Assay
/ Biopsy
/ Cerebrospinal fluid
/ Creutzfeldt-Jakob disease
/ Creutzfeldt-Jakob Syndrome - cerebrospinal fluid
/ Creutzfeldt-Jakob Syndrome - diagnosis
/ Dementia
/ Hospitals
/ Humanities and Social Sciences
/ Humans
/ Lymphoma
/ Medicine
/ multidisciplinary
/ Mutation
/ Neuropathology
/ Pathology
/ Patients
/ Prion Diseases - diagnosis
/ Prion protein
/ Prion Proteins
/ Prions
/ Prions - metabolism
/ Protein seeding
/ Proteins
/ Science
/ Science (multidisciplinary)
/ Skin
/ Skin - metabolism
2024
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Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay
Journal Article
Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay
2024
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Overview
Real-time quaking-induced conversion assay (RT-QuIC) exploits templating activity of pathogenic prion protein for ultrasensitive detection of prions. We have utilized second generation RT-QuIC assay to analyze matching
post-mortem
cerebrospinal fluid and skin samples of 38 prion disease patients and of 30 deceased neurological controls. The analysis of cerebrospinal fluid samples led to 100% sensitivity and 100% specificity, but some samples had to be diluted before the analysis to alleviate the effect of present RT-QuIC inhibitors. The analysis of the corresponding skin samples provided 89.5% sensitivity and 100% specificity. The median seeding dose present in the skin was one order of magnitude higher than in the cerebrospinal fluid, despite the overall fluorescent signal of the skin samples was comparatively lower. Our data support the use of
post-mortem
cerebrospinal fluid for confirmation of prion disease diagnosis and encourage further studies of the potential of skin biopsy samples for
intra-vitam
prion diseases´ diagnostics.
Publisher
Nature Publishing Group UK,Nature Publishing Group,Nature Portfolio
Subject
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