A5004230136- Amyotrophic Lateral Sclerosis Research
- RNA Research and Splicing
- Neurogenetic and Muscular Disorders Research
- Parkinson's Disease Mechanisms and Treatments
- Mitochondrial Function and Pathology
- Genetic Neurodegenerative Diseases
- Alzheimer's disease research and treatments
- Neuroscience and Neuropharmacology Research
- Memory and Neural Mechanisms
- Orthopedic Surgery and Rehabilitation
- Reconstructive Surgery and Microvascular Techniques
- Zeolite Catalysis and Synthesis
- Neurological diseases and metabolism
- Polyomavirus and related diseases
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Inflammatory Bowel Disease
- Regulation of Appetite and Obesity
- Muscle Physiology and Disorders
- Dementia and Cognitive Impairment Research
- Cerebrovascular and genetic disorders
- Fungal Infections and Studies
- Salivary Gland Disorders and Functions
- Glycogen Storage Diseases and Myoclonus
- Surgical site infection prevention
- Sarcoidosis and Beryllium Toxicity Research
Nagoya University
2012-2024
Shiga University
2023-2024
Shiga University of Medical Science
2023-2024
Kyoto University
2024
Shiga Medical Center
2023
Serdang Hospital
2021
Okayama University Hospital
2021
Fukuyama City Hospital
2019-2020
Kurashiki Central Hospital
2011-2019
Fukuyama University
2019
FUS is an RNA-binding protein that regulates transcription, alternative splicing and mRNA transport. Aberrations of are causally associated with familial sporadic ALS/FTLD. We analyzed FUS-mediated transcriptions events in mouse primary cortical neurons using exon arrays. also characterized FUS-binding RNA sites the cerebrum HITS-CLIP. found tend to form stable secondary structures. Analysis position-dependence disclosed scattered binding around alternatively spliced exons including those...
Abstract FUS is an RNA/DNA-binding protein involved in multiple steps of gene expression and associated with amyotrophic lateral sclerosis (ALS) fronto-temporal lobar degeneration (FTLD). However, the specific disease-causing and/or modifying mechanism mediated by largely unknown. Here we evaluate intrinsic roles on synaptic functions animal behaviours. We find that depletion downregulates GluA1, a subunit AMPA receptor. binds GluA1 mRNA vicinity 3′ terminus controls poly (A) tail...
Highlights•The nuclear interaction between FUS and SFPQ is affected by disease mutations•FUS/SFPQ regulate tau isoforms altering skipping of Mapt exon 10•Silencing or in adult mice induces FTLD-like phenotypes•Co-silencing 4R-T rescues the phenotypes FUS/SFPQ-silenced miceSummaryFused sarcoma (FUS) splicing factor, proline- glutamine-rich (SFPQ) are RNA binding proteins that metabolism. We found alternative gene at 10, which generates 4-repeat (4R-T) 3-repeat (3R-T), regulated interactions...
Highlights•FUS regulates post-synaptic density protein interaction and PSD-95 localization•3′UTR length-dependent control of SynGAP α2 by FUS is critical for spine maturation•FUS, together with ELAVL proteins, controls mRNA stability at its 3′UTR•SynGAP supplementation ameliorates behavioral aberrations in FUS-knockout miceSummaryFUS an RNA-binding associated frontotemporal lobar degeneration (FTLD) amyotrophic lateral sclerosis (ALS). Previous reports have demonstrated intrinsic roles...
TDP‐43 and FUS are linked to amyotrophic lateral sclerosis (ALS) frontotemporal lobar degeneration (FTLD), loss of function either protein contributes these neurodegenerative conditions. To elucidate the TDP‐43‐ FUS‐regulated pathophysiological RNA metabolism cascades, we assessed differential gene expression alternative splicing profiles related regulation by or in primary cortical neurons. These overlapped >25% with respect >9% splicing. The shared downstream targets may form a...
FUS is genetically and pathologically linked to amyotrophic lateral sclerosis (ALS) frontotemporal lobar degeneration (FTLD). To clarify the RNA metabolism cascade regulated by in ALS/FTLD, we compared FUS-regulated transcriptome profiles different lineages of primary cells from central nervous system. The FUS-mediated gene expression alternative splicing motor neurons were similar those cortical neurons, but not cerebellar despite similarity innate signature. glial neurons. We identified...
Abstract Alterations of RNA metabolism caused by mutations in RNA-binding protein genes, such as transactivating DNA-binding protein-43 (TDP-43) and fused sarcoma (FUS), have been implicated the pathogenesis amyotrophic lateral sclerosis (ALS). Unlike accumulation TDP43, which is accepted a pathological hall mark sporadic ALS (sALS), FUS pathology sALS still under debate. Although immunoreactive inclusions detected patients previously, technical limitation signal detection, including...
TDP-43 is mislocalized from the nucleus and aggregates within cytoplasm of affected neurons in cases amyotrophic lateral sclerosis. pathology has also been found brain tissues under non-amyotrophic sclerosis conditions, suggesting mechanistic links between TDP-43-related various neurological disorders. This study aimed to assess spinal cord motor tauopathies. We examined 106 cords consecutively autopsied with progressive supranuclear palsy (n = 26), corticobasal degeneration 12), globular...
Fused in sarcoma (FUS) is genetically and clinicopathologically linked to frontotemporal lobar degeneration (FTLD) amyotrophic lateral sclerosis (ALS). We have previously reported that intranuclear interactions of FUS splicing factor, proline- glutamine-rich (SFPQ) contribute neuronal homeostasis. Disruption the FUS-SFPQ interaction leads an increase ratio 4-repeat tau (4R-tau)/3-repeat (3R-tau), which manifests FTLD-like phenotypes mice. Here, we examined 142 autopsied individuals with...
PINK1 and PARKIN are causal genes for hereditary Parkinsonism. Recent studies have shown that Parkin play a pivotal role in the quality control of mitochondria, dysfunction either protein likely results accumulation low‐quality mitochondria triggers early‐onset familial As neurons destined to degenerate /Parkin‐associated Parkinsonism, it is imperative investigate function neurons. However, most investigating /Parkin used non‐neuronal cell lines. Here we show principal cellular events been...
Neuronal intranuclear inclusion disease (NIID) is an uncommon progressive neurodegenerative disorder. Adult-onset NIID can result in prominent dementia. We herein describe the case of a 74-year-old man who presented with dementia, cerebellar ataxia, neuropathy, and autonomic dysfunction. Diffusion-weighted imaging showed hyperintensity corticomedullary junction. Fluid-attenuated inversion recovery images frontal-dominant white matter hyperintensity. was diagnosed from presence inclusions...
Background Although the utility of flaps for treatment sternal wound infections following median sternotomy has been reported 30 years, there have few reports on risk factors complications after reconstruction. The objective this investigation was to identify related reconstruction infections. Methods A retrospective analysis 74 patients with reconstructive surgery infection over a 5-year period performed. Clinical data including age, sex, body mass index (BMI), comorbidities, bacterial...
Alzheimer's disease is a devastating that accompanied by dementia, and its incidence increases with age. However, no interventions have exhibited clear therapeutic effects. We aimed to develop characterize behavioural tasks allow the earlier identification of signs preceding dementia would facilitate development preventative for disease. To this end, we developed 3D virtual reality task sensitive activity grid cells in entorhinal cortex, which region first exhibits neurofibrillary tangles...
We present here a first case of vulvar fibroepithelial stromal polyp (vFSP) appearing in infancy, including previously undescribed papule as an early form vFSP. A 7-month-old girl presented with small (adzuki-sized), soft, erythematous the right labium majus. The size lesion increased up to 2.3 cm following 7 months and exhibited cerebriform or frond-like configuration. surgically removed was characterized by proliferation bland spindle cells tapered cytoplasm, diagnosis vFSP established....
We herein report the case of a 62-year-old man diagnosed with Legionella pneumonia while engaged in recovery work flooded area after Heavy Rain Event July 2018 Japan. The patient was intubated and maintained on mechanical ventilation continuous hemodiafiltration. He also administered antimicrobial therapy ciprofloxacin azithromycin. After 53 days hospital, he discharged. It is important to recognize risk infection take measures prevent it during that involves exposure water soil flood disaster.
We herein report a 75-year-old man with non-small-cell lung cancer who developed tubulointerstitial nephritis due to pembrolizumab administration. He was successfully treated atezolizumab following steroid initially diagnosed adenocarcinoma (T1bN3M1b, stage IV), programmed cell death-ligand 1 tumor proportion score of 25-49%. Although the responded well pembrolizumab, drug discontinued because diagnosis on renal biopsy. Tubulointerstitial 30 mg prednisolone, dose which tapered and maintained...
Phosphorylated and truncated TAR DNA-binding protein-43 (TDP-43) is a major component of ubiquitinated cytoplasmic inclusions in neuronal glial cells two TDP-43 proteinopathies, amyotrophic lateral sclerosis frontotemporal lobar degeneration. Modifications are thus considered to play an important role the pathogenesis proteinopathies. However, both initial cause these abnormal modifications region responsible for its aggregation remain uncertain. Here we report that 32 kDa C-terminal...
An aqueous solution of equimolecular amounts 2-chloropyrimidine and (-)-epigallocatechin-3-O-gallate (EGCg) afforded a colorless block crystal, which was determined to be 2 : complex EGCg by X-ray crystallographic analysis. The formed the cooperative effect three intermolecular interactions, π-π CH-π hydrogen bonds. Upon formation complex, molecule captured hydrophobic space aromatic rings A, B, B' two molecules. molecular capture abilities various heterocyclic compounds using were evaluated...