Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples
Prion disease is an infectious and fatal neurodegenerative disease. Western blotting (WB)-based identification of proteinase K (PK)-resistant prion protein (PrPres) is considered a definitive diagnosis of prion diseases. In this study, we aimed to detect PrPres using formalin-fixed paraffin-embedded...
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2024-12-01
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| Series: | Prion |
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| Online Access: | https://www.tandfonline.com/doi/10.1080/19336896.2024.2337981 |
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| author | Sachiko Koyama Kaoru Yagita Hideomi Hamasaki Hideko Noguchi Masahiro Shijo Kosuke Matsuzono Kei-Ichiro Takase Keita Kai Shin-Ichi Aishima Kyoko Itoh Toshiharu Ninomiya Naokazu Sasagasako Hiroyuki Honda |
| author_facet | Sachiko Koyama Kaoru Yagita Hideomi Hamasaki Hideko Noguchi Masahiro Shijo Kosuke Matsuzono Kei-Ichiro Takase Keita Kai Shin-Ichi Aishima Kyoko Itoh Toshiharu Ninomiya Naokazu Sasagasako Hiroyuki Honda |
| author_sort | Sachiko Koyama |
| collection | DOAJ |
| description | Prion disease is an infectious and fatal neurodegenerative disease. Western blotting (WB)-based identification of proteinase K (PK)-resistant prion protein (PrPres) is considered a definitive diagnosis of prion diseases. In this study, we aimed to detect PrPres using formalin-fixed paraffin-embedded (FFPE) specimens from cases of sporadic Creutzfeldt–Jakob disease (sCJD), Gerstmann–Sträussler–Scheinker disease (GSS), glycosylphosphatidylinositol-anchorless prion disease (GPIALP), and V180I CJD. FFPE samples were prepared after formic acid treatment to inactivate infectivity. After deparaffinization, PK digestion was performed, and the protein was extracted. In sCJD, a pronounced PrPres signal was observed, with antibodies specific for type 1 and type 2 PrPres exhibited a strong or weak signals depending on the case. Histological examination of serial sections revealed that the histological changes were compatible with the biochemical characteristics. In GSS and GPIALP, prion protein core-specific antibodies presented as PrPres bands at 8–9 kDa and smear bands, respectively. However, an antibody specific for the C-terminus presented as smears in GSS, with no PrPres detected in GPIALP. It was difficult to detect PrPres in V180I CJD. Collectively, our findings demonstrate the possibility of detecting PrPres in FFPE and classifying the prion disease types. This approach facilitates histopathological and biochemical evaluation in the same sample and is safe owing to the inactivation of infectivity. Therefore, it may be valuable for the diagnosis and research of prion diseases. |
| format | Article |
| id | doaj-art-88736088322f420ebc3eef6459d81384 |
| institution | Kabale University |
| issn | 1933-6896 1933-690X |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Prion |
| spelling | doaj-art-88736088322f420ebc3eef6459d813842025-02-05T12:40:52ZengTaylor & Francis GroupPrion1933-68961933-690X2024-12-01181405310.1080/19336896.2024.2337981Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samplesSachiko Koyama0Kaoru Yagita1Hideomi Hamasaki2Hideko Noguchi3Masahiro Shijo4Kosuke Matsuzono5Kei-Ichiro Takase6Keita Kai7Shin-Ichi Aishima8Kyoko Itoh9Toshiharu Ninomiya10Naokazu Sasagasako11Hiroyuki Honda12Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDepartment of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDepartment of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDepartment of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDepartment of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDivision of Neurology, Department of Medicine, Jichi Medical University, Tochigi, JapanDepartment of Neurology, Aso Iizuka Hospital, Fukuoka, JapanDepartment of Pathology, Saga University Hospital, Saga, JapanDepartment of Scientific Pathology Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDepartment of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, JapanDepartment of Epidemiology and Public Health, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanDepartment of Neurology, Neuro-Muscular Center, National Hospital Organization, Omuta National Hospital, Fukuoka, JapanDepartment of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanPrion disease is an infectious and fatal neurodegenerative disease. Western blotting (WB)-based identification of proteinase K (PK)-resistant prion protein (PrPres) is considered a definitive diagnosis of prion diseases. In this study, we aimed to detect PrPres using formalin-fixed paraffin-embedded (FFPE) specimens from cases of sporadic Creutzfeldt–Jakob disease (sCJD), Gerstmann–Sträussler–Scheinker disease (GSS), glycosylphosphatidylinositol-anchorless prion disease (GPIALP), and V180I CJD. FFPE samples were prepared after formic acid treatment to inactivate infectivity. After deparaffinization, PK digestion was performed, and the protein was extracted. In sCJD, a pronounced PrPres signal was observed, with antibodies specific for type 1 and type 2 PrPres exhibited a strong or weak signals depending on the case. Histological examination of serial sections revealed that the histological changes were compatible with the biochemical characteristics. In GSS and GPIALP, prion protein core-specific antibodies presented as PrPres bands at 8–9 kDa and smear bands, respectively. However, an antibody specific for the C-terminus presented as smears in GSS, with no PrPres detected in GPIALP. It was difficult to detect PrPres in V180I CJD. Collectively, our findings demonstrate the possibility of detecting PrPres in FFPE and classifying the prion disease types. This approach facilitates histopathological and biochemical evaluation in the same sample and is safe owing to the inactivation of infectivity. Therefore, it may be valuable for the diagnosis and research of prion diseases.https://www.tandfonline.com/doi/10.1080/19336896.2024.2337981FFPEPK-resistant prion proteinPrion diseaseSporadic Creutzfeldt–Jakob diseasewestern blotting |
| spellingShingle | Sachiko Koyama Kaoru Yagita Hideomi Hamasaki Hideko Noguchi Masahiro Shijo Kosuke Matsuzono Kei-Ichiro Takase Keita Kai Shin-Ichi Aishima Kyoko Itoh Toshiharu Ninomiya Naokazu Sasagasako Hiroyuki Honda Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples Prion FFPE PK-resistant prion protein Prion disease Sporadic Creutzfeldt–Jakob disease western blotting |
| title | Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples |
| title_full | Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples |
| title_fullStr | Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples |
| title_full_unstemmed | Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples |
| title_short | Novel method for classification of prion diseases by detecting PrPres signal patterns from formalin-fixed paraffin-embedded samples |
| title_sort | novel method for classification of prion diseases by detecting prpres signal patterns from formalin fixed paraffin embedded samples |
| topic | FFPE PK-resistant prion protein Prion disease Sporadic Creutzfeldt–Jakob disease western blotting |
| url | https://www.tandfonline.com/doi/10.1080/19336896.2024.2337981 |
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