A5052000092- Prion Diseases and Protein Misfolding
- Neurological diseases and metabolism
- Alcoholism and Thiamine Deficiency
- Cardiac Imaging and Diagnostics
- Trace Elements in Health
- Mesenchymal stem cell research
- Infectious Encephalopathies and Encephalitis
- Neurological Disease Mechanisms and Treatments
- Cerebrovascular and Carotid Artery Diseases
- Alzheimer's disease research and treatments
- T-cell and Retrovirus Studies
- Parkinson's Disease Mechanisms and Treatments
- Fibromyalgia and Chronic Fatigue Syndrome Research
- RNA regulation and disease
- Neuroinflammation and Neurodegeneration Mechanisms
- Head and Neck Cancer Studies
- Amyotrophic Lateral Sclerosis Research
- Myasthenia Gravis and Thymoma
- Medical Imaging Techniques and Applications
- Cancer Diagnosis and Treatment
- Intracranial Aneurysms: Treatment and Complications
- Neurological disorders and treatments
- Vector-Borne Animal Diseases
- Monoclonal and Polyclonal Antibodies Research
- Amino Acid Enzymes and Metabolism
Nagasaki University
2016-2025
Department of Medical Sciences
2022-2025
Japan Pediatric Society
2024
Doshisha University
2023
Nagasaki Ajisai Hospital
2006-2022
Tohoku University
2002-2022
National Hospital Organization
2022
Chiba University
1983-2022
Hokkaido University
1991-2020
Satou Hospital
2012-2017
Real-time quaking-induced conversion (RT-QuIC) allows the amplification of miniscule amounts scrapie prion protein (PrP(Sc)). Recent studies applied RT-QuIC methodology to cerebrospinal fluid (CSF) for diagnosing human diseases. However, date, there has not been a formal multi-centre assessment reproducibility, validity and stability in this context, an indispensable step establishment as diagnostic test clinical practice. In present study, we analysed CSF from 110 disease patients 400...
We recently developed a new in vitro amplification technology, designated “real-time quaking-induced conversion (RT-QUIC)”, for detection of the abnormal form prion protein (PrPSc) easily accessible specimens such as cerebrospinal fluid (CSF). After assessment more than 200 CSF from Japanese and Australian patients, we found no instance false positive, 80% accuracy correct diagnosis sporadic Creutzfeldt–Jakob disease (sCJD). Furthermore, RT-QUIC can be applied to other diseases, including...
Introduction The definitive diagnosis of genetic prion diseases (gPrD) requires pathological confirmation. To date, has relied upon the finding biomarkers 14-3-3 protein and total tau (t-tau) in cerebrospinal fluid (CSF), but many researchers have reported that these markers are not sufficiently elevated gPrD, especially Gerstmann-Sträussler-Scheinker syndrome (GSS). We recently developed a new vitro amplification technology, designated "real-time quaking-induced conversion (RT-QUIC)", to...
Real-time quaking-induced conversion (RT-QuIC) has been proposed as a sensitive diagnostic test for sporadic Creutzfeldt-Jakob disease; however, before this assay can be introduced into clinical practice, its reliability and reproducibility need to demonstrated. Two international ring trials were undertaken in which set of 25 cerebrospinal fluid samples analyzed by total 11 different centers using range recombinant prion protein substrates instrumentation. The results show almost complete...
Background and Purpose— Intra-arterial cell transplantation offers a novel therapeutic strategy for stroke; however, it remains unclear how the timing of administration affects distribution, brain repair processes, functional recovery. Here, we investigate hypothesis that changes behavior graft host environment in way Methods— Rats received human mesenchymal stem cells via internal carotid artery at 1, 4, or 7 days (D1, D4, D7) after middle cerebral occlusion reperfusion. Animals were...
Prion diseases are fatal neurodegenerative disorders and no effective treatment has been established to date. In this study, we evaluated the effect of FK506 (tacrolimus), a macrolide that is known be mild immunosuppressant, on prion infection, using cell culture animal models. We found markedly reduced abnormal form protein (PRNPSc) in cultures (N2a58 MG20) infected with Fukuoka-1 prion. The levels autophagy-related molecules such as LC3-II, ATG12–ATG5 ATG7 were significantly increased...
The prion-like seeding of misfolded α-synuclein (αSyn) involved in the pathogenesis Lewy body diseases (LBD) remains poorly understood at molecular level. Using real-time quaking-induced conversion (RT-QUIC) assay, we investigated whether brain tissues from cases dementia with bodies (DLB), which contain serine 129 (Ser129)-phosphorylated insoluble aggregates αSyn, can convert Escherichia coli-derived recombinant αSyn (r-αSyn) to fibrils. Diffuse neocortical DLB yielded 50% dose (SD50)...
Objective. We evaluated the significance of power Doppler ultrasonography (PDUS) score by comparing it with serum biomarkers and clinical disease activity.
The objective was to characterize a rapidly progressive subtype of Alzheimer's disease (rpAD). Multicenter (France, Germany, Japan, Spain) retrospective analyses neuropathologically confirmed rpAD cases initially classified as prion due their clinical phenotype were performed. Genetic properties, cerebrospinal fluid biomarkers, neuropathology, and features examined. Eighty-nine patients included (median survival 10 months). APOE PRNP codon 129 genotype distribution paralleled healthy control...
Abstract Prion diseases are fatal neurodegenerative disorders characterized by the accumulation of prion protein (PrP C ). To date, there is no effective treatment for disease. The accumulated PrP, termed PrP Sc , forms amyloid fibrils and could be infectious. It has been suggested that abnormally folded resistant to proteolytic degradation also inhibits proteasomal functions in infected cells, thereby inducing neuronal death. Recent work indicates ubiquitin-proteasome system involved...
The ubiquitin‒proteasome system (UPS) and autophagy are the two primary cellular pathways of misfolded or damaged protein degradation that maintain proteostasis. When proteasome is dysfunctional, cells compensate for impaired clearance by activating aggrephagy, a type selective autophagy, to eliminate ubiquitinated aggregates; however, molecular mechanisms which function activates aggrephagy remain poorly understood. Here, we demonstrate activation transcriptionally induced transcription...
To study the asymmetry in signal intensity of sigmoid sinuses, internal jugular veins (IJVs), or both sets structures on magnetic resonance (MR) images, authors reviewed 226 serial routine MR imaging studies. Cerebral digital subtraction angiography was performed 20 patients with a markedly higher and/or enhancement left; 15 them also underwent venography. With every sequence, left sinus, IJV, were significantly more often than right (range, P = .0001 to .0129). Angiography revealed...
We detected deposits of IgG, C3, and C9 (immune complexes) at the limb muscle motor end-plates (biceps brachii muscle) in 16 19 patients who exhibited only ocular signs symptoms myasthenia gravis that were improved by intravenous injections edrophonium chloride. Circulating anti-acetylcholine receptor (anti-AChR) antibodies negative 6 patients, but end-plate fine structure postsynaptic regions was abnormal all 16. Single-fiber EMG revealed no abnormalities 8 13 studied. Our results indicate...
Prion diseases are neurodegenerative disorders characterized by the aggregation of abnormally folded prion protein (PrPSc). In this study, we focused on mechanism clearance PrPSc, which remains unclear. p62 is a cytosolic known to mediate both formation and degradation aggregates abnormal proteins. The levels increased in prion-infected brains persistently infected cell cultures. Upon proteasome inhibition, co-localized with forming large aggregate perinuclear region, hereafter referred as...
Objectives Genetic Creutzfeldt-Jakob disease (CJD) due to V180I mutation in the prion protein gene ( PRNP ) is of great interest because differences from sporadic CJD and other genetic diseases terms clinical features, as well pathological biochemical findings. However, few systematic observations about features patients with this unique have been published. Therefore, goal study was relate forms a perspective. Design We analysed symptoms, genetics, biomarkers cerebrospinal fluid (CSF) MRI...
Cell transplantation therapy offers great potential to improve impairments after stroke. However, the importance of donor age on therapeutic efficacy is unclear. We investigated regenerative capacity transplanted cells focusing (young vs. old) for ischaemic The quantities human mesenchymal stem cell (hMSC) secreted brain-derived neurotrophic factor in vitro and monocyte chemotactic protein-1 at day 7 vivo were both significantly higher young hMSC compared with old hMSC. Male Sprague-Dawley...
<i>Background:</i> We have recently begun to doubt the effectiveness of periodic sharp wave complexes observed on electroencephalographs and detection 14-3-3 protein in cerebrospinal fluid (CSF) as diagnostic criteria for Creutzfeldt-Jakob disease (CJD). Diffusion-weighted magnetic resonance imaging (DWI) total tau (t-tau) CSF may be more sensitive criteria. <i>Methods:</i> Among 44 CJD patients, we selected 21 subjects that suffered from early-stage CJD, which was...
A national system for surveillance of prion diseases (PrDs) was established in Japan April 1999. Here, we analyzed the relationships among protein gene (PRNP) mutations and clinical features, cerebrospinal fluid (CSF) markers, pathological characteristics major genotypes genetic PrDs (gPrDs). We retrospectively age at onset disease duration; concentrations incidences 14-3-3 protein, tau abnormal (PrPSc) CSF 309 gPrD patients with P102L, P105L, E200K, V180I, or M232R mutations; brain...
ABSTRACT The phenomenon of prion strains with distinct biological characteristics has been hypothesized to be involved in the structural diversity abnormal protein (PrP Sc ). However, molecular basis transmission strain properties remains poorly understood. Real-time quaking-induced conversion (RT-QUIC) is a cell-free system that uses Escherichia coli -derived recombinant PrP (rPrP) for sensitive detection . To investigate whether various can transmitted amyloid fibrils consisting rPrP (rPrP...