Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease

Prion diseases, including Creutzfeldt–Jakob disease (CJD), are deadly neurodegenerative disorders characterized by the buildup of abnormal prion proteins in the brain. This accumulation disrupts neuronal functions, leading to the rapid onset of psychiatric symptoms, ataxia, and cognitive decline. Th...

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Main Authors: Mika Shimamura, Kong Weijie, Toshiaki Nonaka, Koki Kosami, Ryusuke Ae, Koji Fujita, Taiki Matsubayashi, Tadashi Tsukamoto, Nobuo Sanjo, Katsuya Satoh
Format: Article
Language:English
Published: MDPI AG 2024-12-01
Series:Biomolecules
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Online Access:https://www.mdpi.com/2218-273X/15/1/8
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author Mika Shimamura
Kong Weijie
Toshiaki Nonaka
Koki Kosami
Ryusuke Ae
Koji Fujita
Taiki Matsubayashi
Tadashi Tsukamoto
Nobuo Sanjo
Katsuya Satoh
author_facet Mika Shimamura
Kong Weijie
Toshiaki Nonaka
Koki Kosami
Ryusuke Ae
Koji Fujita
Taiki Matsubayashi
Tadashi Tsukamoto
Nobuo Sanjo
Katsuya Satoh
author_sort Mika Shimamura
collection DOAJ
description Prion diseases, including Creutzfeldt–Jakob disease (CJD), are deadly neurodegenerative disorders characterized by the buildup of abnormal prion proteins in the brain. This accumulation disrupts neuronal functions, leading to the rapid onset of psychiatric symptoms, ataxia, and cognitive decline. The urgency of timely diagnosis for effective treatment necessitates the identification of strongly correlated biomarkers in bodily fluids, which makes our research crucial. In this study, we employed a fully automated multiplex ELISA (Ella<sup>®</sup>) to measure the concentrations of 14-3-3 protein, total tau protein, and neurofilament light chain (NF-L) in cerebrospinal fluid (CSF) and serum samples from patients with prion disease and analyzed their link to disease prognosis. However, in North American and European cases, we did not confirm a correlation between NF-L levels and survival time. This discrepancy is believed to stem from differences in treatment policies and measurement methods between Japan and the United States. Nonetheless, our findings suggest that NF-L concentrations could be an early diagnostic marker for CJD patients with further enhancements. The potential impact of our findings on the early diagnosis of CJD patients is significant. Future research should focus on increasing the number of sCJD cases studied in Japan and gathering additional evidence using next-generation measurement techniques.
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spelling doaj-art-68ecb7ec15a34a06899fadcadb16e3cc2025-01-24T13:24:50ZengMDPI AGBiomolecules2218-273X2024-12-01151810.3390/biom15010008Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion DiseaseMika Shimamura0Kong Weijie1Toshiaki Nonaka2Koki Kosami3Ryusuke Ae4Koji Fujita5Taiki Matsubayashi6Tadashi Tsukamoto7Nobuo Sanjo8Katsuya Satoh9Biomedical Research Support Center, Nagasaki University, 1-12-4 Sakamoto, Nagasaki 852-8523, JapanUnit of Medical and Dental Sciences, Department of Health Sciences, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki 852-8523, JapanDivision of Cellular and Molecular Biology, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki 852-8523, JapanDivision of Public Health, Center for Community Medicine, Jichi Medical University, Tochigi 329-0498, JapanDivision of Public Health, Center for Community Medicine, Jichi Medical University, Tochigi 329-0498, JapanDepartment of Neurology, Tokushima University Graduate School of Biomedical Sciences, 3-18-15 Kuramoto-cho, Tokushima 770-8503, JapanDepartment of Neurology and Neurological Science, Tokyo Medical and Dental University, Graduate School of Medical and Dental Sciences, 1-5-45 Yushima Bunkyo-ku, Tokyo 113-8510, JapanDepartment of Neurology, National Center of Neurology and Psychiatry (NCNP), 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8551, JapanDepartment of Internal Medicine, Division of Neurology, Kudanzaka Hospital, 1-6-12 Kudan-minami, Chiyoda-ku, Tokyo 102-0074, JapanUnit of Medical and Dental Sciences, Department of Health Sciences, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki 852-8523, JapanPrion diseases, including Creutzfeldt–Jakob disease (CJD), are deadly neurodegenerative disorders characterized by the buildup of abnormal prion proteins in the brain. This accumulation disrupts neuronal functions, leading to the rapid onset of psychiatric symptoms, ataxia, and cognitive decline. The urgency of timely diagnosis for effective treatment necessitates the identification of strongly correlated biomarkers in bodily fluids, which makes our research crucial. In this study, we employed a fully automated multiplex ELISA (Ella<sup>®</sup>) to measure the concentrations of 14-3-3 protein, total tau protein, and neurofilament light chain (NF-L) in cerebrospinal fluid (CSF) and serum samples from patients with prion disease and analyzed their link to disease prognosis. However, in North American and European cases, we did not confirm a correlation between NF-L levels and survival time. This discrepancy is believed to stem from differences in treatment policies and measurement methods between Japan and the United States. Nonetheless, our findings suggest that NF-L concentrations could be an early diagnostic marker for CJD patients with further enhancements. The potential impact of our findings on the early diagnosis of CJD patients is significant. Future research should focus on increasing the number of sCJD cases studied in Japan and gathering additional evidence using next-generation measurement techniques.https://www.mdpi.com/2218-273X/15/1/8neurofilament light chainprionCreutzfeldt–Jakob diseaseserumcerebrospinal fluid
spellingShingle Mika Shimamura
Kong Weijie
Toshiaki Nonaka
Koki Kosami
Ryusuke Ae
Koji Fujita
Taiki Matsubayashi
Tadashi Tsukamoto
Nobuo Sanjo
Katsuya Satoh
Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease
Biomolecules
neurofilament light chain
prion
Creutzfeldt–Jakob disease
serum
cerebrospinal fluid
title Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease
title_full Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease
title_fullStr Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease
title_full_unstemmed Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease
title_short Neurofilament Light Chain Levels in Serum and Cerebrospinal Fluid Do Not Correlate with Survival Times in Patients with Prion Disease
title_sort neurofilament light chain levels in serum and cerebrospinal fluid do not correlate with survival times in patients with prion disease
topic neurofilament light chain
prion
Creutzfeldt–Jakob disease
serum
cerebrospinal fluid
url https://www.mdpi.com/2218-273X/15/1/8
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