A5082495125- Neurological diseases and metabolism
- Glioma Diagnosis and Treatment
- Parkinson's Disease Mechanisms and Treatments
- Prion Diseases and Protein Misfolding
- Autophagy in Disease and Therapy
- Genetic Neurodegenerative Diseases
- Hedgehog Signaling Pathway Studies
- Alcoholism and Thiamine Deficiency
- Amyotrophic Lateral Sclerosis Research
- Mitochondrial Function and Pathology
- Alzheimer's disease research and treatments
- Virology and Viral Diseases
- Epigenetics and DNA Methylation
- Neurogenetic and Muscular Disorders Research
- MicroRNA in disease regulation
- Epilepsy research and treatment
- Genetics and Neurodevelopmental Disorders
- Neurological disorders and treatments
- Metabolism and Genetic Disorders
- ATP Synthase and ATPases Research
- Trace Elements in Health
- Asthma and respiratory diseases
- Chromatin Remodeling and Cancer
- RNA regulation and disease
- Lipid metabolism and biosynthesis
Aichi Medical University
2018-2025
Niigata University
2010-2021
Oita University
2010-2020
Johns Hopkins University
2015-2019
Johns Hopkins Medicine
2017-2019
Johns Hopkins Hospital
2015-2016
Sumitomo Mitsui Banking Corporation
1996
Ibaraki University
1988
Hitachi (Japan)
1988
Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative characterized by the presence of inclusions unknown origin. NIID caused an expansion GGC repeats in 5′ UTR NOTCH2NLC (N2C) gene. We found that these are embedded small upstream open reading frame (uORF) (uN2C), resulting their translation into polyglycine-containing protein, uN2CpolyG. This protein accumulates cell and mouse models tissue samples individuals with NIID. Furthermore, expression uN2CpolyG mice leads to...
Subacute sclerosing panencephalitis (SSPE) occurs in some individuals after measles infection, following a symptom-free period of several years. It resembles chronic traumatic encephalopathy (CTE), which happens repetitive head impacts or exposure to blast waves, period. As CTE, the neurofibrillary changes SSPE are concentrated superficial cortical layers. Here we used electron cryo-microscopy (cryo-EM) tau filaments from two cases show that folds and CTE identical. Two types were each made...
Autophagy is a dynamic process of protein degradation. Induction autophagy by temozolomide (TMZ) has been noted in glioma cell lines. Twenty‐eight specimens, obtained from 14 patients before and after TMZ treatment, were analyzed to investigate whether induction could be detected surgical specimens immunohistochemical analysis. Macroautophagy was monitored analysis employing anti‐light chain 3 isoform B (LC3B) anti‐lysosome‐associated membrane 1 (LAMP1) antibodies; chaperone‐mediated...
Abstract Glioblastomas are highly aggressive tumors that contain treatment resistant stem‐like cells. Therapies targeting developmental pathways such as Notch eliminate many neoplastic glioma cells, including those with stem cell features, but their efficacy can be limited by various mechanisms. One potential avenue for chemotherapeutic resistance is the induction of autophagy, little known how it might modulate response to inhibitors. We used γ‐secretase inhibitor MRK003 block pathway...
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...
Subacute sclerotic panencephalitis (SSPE) is a refractory neurological disorder after exposure to measles virus. Recently, SSPE cases have been treated with antiviral therapies, but data on the efficacy are inconclusive. Abnormal tau accumulation has reported in brain tissue of cases, there few reports which this amply discussed. Five autopsied diagnosed as definite were included study. The subject age or disease duration ranged from 7.6 40.9 years old 0.5 20.8 years, respectively. Cases 3...
Abstract The striatonigral and olivopontocerebellar systems are known to be vulnerable in multiple system atrophy (MSA), showing neuronal loss, astrogliosis, alpha‐synuclein‐immunoreactive inclusions. MSA patients who displayed abundant cytoplasmic inclusions (NCIs) the regions other than or have occasionally been diagnosed with variants of MSA. In this study, we report clinical pathologic findings characterized by prominent involvement hippocampus. We assessed 146 consecutively autopsied...
Cite this as: H. Miyahara, N. Okazaki,T. Nagakura, S. Korematsu and T. Izumi,Clinical & Experimental Allergy, 2011 (41) 186–191. Summary Background Thymus‐and‐activation‐regulated chemokine (TARC; CCL17) is related to both allergy pregnancy, but the relationships of maternal umbilical cord blood CCL17 atopic dermatitis (AD) development have not yet been examined. Objective Seventy paired full‐term normal vaginal delivery newborns their mothers were enrolled in study. Methods To elucidate...
The transactive response DNA-binding protein of 43 kDa (TDP-43) is a pathological amyotrophic lateral sclerosis (ALS). TDP-43 pathology characterized by combination the cytoplasmic aggregation and nuclear clearance this protein. However, mechanisms underlying have not been fully clarified. aim study was to evaluate relationships between expression level properties aggregates in autopsied ALS cases. We included 22 consecutively cases with sporadic TDP-43-related ALS. motor neuron systems were...
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor that usually occurs in the superficial cerebral hemispheres of children and young adults has relatively favorable prognosis. We report an unusual case supratentorial, intraventricular 52-year-old man. The was composed pleomorphic cells, including giant most which were multinucleated, small cells. In addition, frequent xanthic changes cytoplasm widespread reticulin deposits lymphocytic infiltrates stroma characteristic features....
Intensive chemotherapeutic regimens with craniospinal irradiation have greatly improved survival in medulloblastoma patients. However, markedly differs among molecular subgroups and their biomarkers are unknown. Through unbiased screening, we found Schlafen family member 11 (SLFN11), which is known to improve response DNA damaging agents various cancers, be one of the top prognostic markers medulloblastomas. Hence, explored expression functions SLFN11 medulloblastoma.SLFN11 for each subgroup...
The central nervous system (CNS) inflammation of Kawasaki disease (KD) has not been sufficiently evaluated in spite the complications irritability and CSF pleocytosis.Cerebrospinal fluid (CSF) serum inflammatory cytokine values were simultaneously examined 10 patients (2.6 +/- 2.1 year age) during acute phase. They all irritable demonstrated mild consciousness disturbance.The IL6 was elevated (>3.0 pg/mL) 6 patients, 4 them showed higher than values. sTNFR1 (>0.5 microg/mL) 1 These (IL6;...
Abstract The retinal pathology of genetically confirmed neuronal intranuclear inclusion disease (NIID) is yet unknown. We report the ocular findings in four NIID patients with NOTCH2NLC GGC repeat expansion to investigate retinopathy. All were diagnosed by skin biopsy and analysis. Ocular studied using fundus photographs, optical coherence tomographic images (OCT), full-field electroretinograms (ERGs). histopathology retina was on autopsy samples from two cases immunohistochemistry. had an...
Abstract Background: To elucidate the basic mechanism of theophylline‐associated seizures (TAS), clinical symptoms, electroencephalogram (EEG) and neuroradiological imaging eight pediatric patients were all retrospectively evaluated. Methods: Patients whose represented their first episode selected, while with cerebrospinal fluid abnormalities including pleocytosis protein elevation, present illness head trauma, epilepsy, febrile convulsion or any psychomotor retardation excluded although...
Korematsu S, Yamamoto K, Nagakura T, Miyahara H, Okazaki N, Akiyoshi Maeda Suenobu S‐i, Izumi T. The indication and effectiveness of low‐dose erythromycin therapy in pediatric patients with bronchial asthma. Pediatr Allergy Immunol 2010: 21: 489–492. © 2010 John Wiley & Sons A/S To elucidate the mechanisms intractable asthma (EM) therapy, serum chemokine levels angiogenic factor were evaluated 55 asthma; 7.4 ± 3.5 yr old, who had been treated inhaled steroid, leukotriene receptor...
Hypertrophic and dysmorphic neurons have been identified in the hippocampal end folium of patients with mesial temporal lobe epilepsy (mTLE). No data are available regarding correlation between these cellular alterations severity sclerosis (HS), significance this phenomenon has unclear. We evaluated both perikaryon nuclear areas residual 47 mTLE, seven lesional neocortical (LTLE), 10 controls without seizure episodes. According to neuron loss folium, we defined mTLE cases showing slight...
Tuberous sclerosis complex (TSC) is characterized by benign tumors and hamartomas, including cortical tubers. Hamartin tuberin, encoded the TSC 1 2 genes, respectively, constitute a functional that negatively regulates mammalian target of rapamycin (mTOR) signaling pathway, eventually promoting induction autophagy. In present study, we assessed autophagy in tubers surgically removed from seven patients with comparison five controls tissue taken non-TSC epilepsy. Immunoblotting demonstrated...
The interaction of matrix metalloproteinase (MMP)-9 and tissue inhibitor metalloproteinase-1 has been implicated in the formation coronary aneurysms Kawasaki disease. MMP-9 were distributed predominantly granulocytes platelets, respectively, patients with plasma values correlated positively circulating neutrophil count. Inhibiting activity maintaining platelet might prevent aneurysms.
Medulloblastoma ( MB ) is a malignant cerebellar tumor arising in children, and its ontogenesis regulated by S onic H edgehog Shh signaling. No data are available regarding the correlation between expression of Gli3 , protein lying downstream hh, neuronal differentiation cells, or prognostic significance these features. We re‐evaluated histopathological features surgical specimens taken from 32 patients, defined 15 them as with ND ), three both glial GD 14 differentiation‐free DF ....