A5069538041- Pluripotent Stem Cells Research
- Neurological disorders and treatments
- Glycogen Storage Diseases and Myoclonus
- Alzheimer's disease research and treatments
- Genetics and Neurodevelopmental Disorders
- Amyotrophic Lateral Sclerosis Research
- Autoimmune Neurological Disorders and Treatments
- Atmospheric chemistry and aerosols
- CRISPR and Genetic Engineering
- Hepatocellular Carcinoma Treatment and Prognosis
- Multiple Sclerosis Research Studies
- Parkinson's Disease Mechanisms and Treatments
- Peripheral Neuropathies and Disorders
- Neurogenetic and Muscular Disorders Research
- Liver Disease Diagnosis and Treatment
- RNA regulation and disease
- Plant responses to elevated CO2
- Advanced materials and composites
- Neurogenesis and neuroplasticity mechanisms
- T-cell and B-cell Immunology
- RNA Research and Splicing
- Electrocatalysts for Energy Conversion
- Retinal Development and Disorders
- Advanced ceramic materials synthesis
- Photosynthetic Processes and Mechanisms
Chiba University
2021-2025
Kansai Medical University
2019-2025
University Medical Center
2025
RIKEN Center for Advanced Intelligence Project
2019-2024
Kyoto University
2015-2024
RIKEN BioResource Research Center
2017-2024
Tokyo Metropolitan University
2024
Japan Science and Technology Agency
2013-2023
Chiba University Hospital
2023
Kyushu Institute of Technology
2019-2022
Anacardic acid attenuates mutant TDP-43–associated abnormalities in motor neurons derived from ALS patient–specific induced pluripotent stem cells.
Analysis of ALS patient iPSC-derived motor neurons indicates that Src/c-Abl inhibitors may have potential for treating ALS.
The potential use of induced pluripotent stem cells (iPSCs) in personalized regenerative medicine applications may be augmented by transgenics, including the expression constitutive cell labels, differentiation reporters, or modulators disease phenotypes. Thus, there is precedence for reproducible transgene amongst iPSC sub-clones with isogenic diverse genetic backgrounds. Using virus transposon vectors, integration sites and copy numbers are difficult to control, nearly impossible reproduce...
Multiple sclerosis (MS) is a T cell-mediated autoimmune disease of the central nervous system. Foxp3+ regulatory (Treg) cells are reduced in frequency and dysfunctional patients with MS, but underlying mechanisms this deficiency unclear. Here, we show that induction human IFN-γ-IL-17A-Foxp3+CD4+ inhibited presence circulating exosomes from MS. The exosomal miRNA profile MS differs healthy controls, let-7i, which markedly increased suppresses Treg by targeting insulin like growth factor 1...
Highlights•Rapid, robust neuronal induction from human iPSCs to model AD drug responsiveness•iPSC-based screening of pharmaceutical compounds for Aβ phenotypes•A combination existing drugs synergistically improve phenotypes AD•Anti-Aβ cocktail decreases toxic levels in neurons derived patients' cellsSummaryIn the process development, vitro studies do not always adequately predict human-specific responsiveness clinical trials. Here, we applied advantage iPSC-derived neurons, which offer...
Transplantation of glial-rich neural progenitors has been demonstrated to attenuate motor neuron degeneration and disease progression in rodent models mutant superoxide dismutase 1 (SOD1)-mediated amyotrophic lateral sclerosis (ALS). However, translation these results into a clinical setting requires renewable human cell source. Here, we derived from iPSCs transplanted them the lumbar spinal cord ALS mouse models. The cells differentiated astrocytes, treated group showed prolonged lifespan....
Inherited retinal dystrophies (IRDs) are progressive diseases leading to vision loss. Mutation in the eyes shut homolog (EYS) gene is one of most frequent causes IRD. However, mechanism photoreceptor cell degeneration by mutant EYS has not been fully elucidated. Here, we generated organoids from induced pluripotent stem cells (iPSCs) derived patients with EYS-associated dystrophy (EYS-RD). In RD organoids, both and G protein–coupled receptor kinase 7 (GRK7), proteins handling phototoxicity,...
Although CD1d‐restricted NKT cells have been implicated as a participant in the regulatory mechanism of autoimmune diseases, it remains unclear how they would regulate human diseases such multiple sclerosis (MS). Furthermore, although comprise CD4+ and CD4– populations, prior studies often represented them simply population. Given that may represent functionally distinct appears crucial to examine individual subset diseases. Here we studied frequency cytokine phenotypes fresh peripheral...
Abstract Mutations in the gene MAPT encoding tau, a microtubules-associated protein, cause subtype of familial neurodegenerative disorder, known as frontotemporal lobar degeneration tauopathy (FTLD-Tau), which presents with dementia and is characterized by atrophy frontal temporal lobes brain. Although induced pluripotent stem cell (iPSC) technology has facilitated investigation phenotypes FTLD-Tau patient neuronal cells vitro , it remains unclear how neurons degenerate. Here, we established...
The blood-brain barrier (BBB) is composed of four cell populations, brain endothelial cells (BECs), pericytes, neurons, and astrocytes. Its role to precisely regulate the microenvironment through selective substance crossing. Here we generated an in vitro model BBB by differentiating human induced pluripotent stem (hiPSCs) into all populations. When hiPSC-derived populations were co-cultured, (ECs) endowed with features consistent BECs, including a high expression nutrient transporters...
Abstract Aim This study aimed to evaluate the safety and efficacy of durvalumab plus tremelimumab in patients with advanced hepatocellular carcinoma who have previously received atezolizumab bevacizumab (Atez/Bev). Additionally, it seeks assess feasibility administering immunotherapy after occurrence immune‐mediated adverse events (imAEs) real‐world clinical practice. Methods retrospective analyzed data from treated at four Japanese institutions. Clinical outcomes, events, tumor dynamics,...
ABSTRACT Aim Combination immunotherapy is the standard of care for advanced hepatocellular carcinoma (HCC). However, some patients are unsuitable such treatment. This study investigated safety and effectiveness durvalumab monotherapy in a real‐world cohort with HCC who were poor candidates combination immunotherapy. Methods We retrospectively analyzed data from 35 treated across three Japanese institutions between January December 2023. Patients selected based on their ineligibility or...
Neuromyelitis optica (NMO) is an acute inflammatory demyelinating disease of the CNS. The presence astrocyte-targeted AQP4-immunoglobulin G (IgG) in peripheral blood a major factor its diagnosis. Previous studies show that AQP4-IgG directly contributes to CNS inflammation, and B cells play central pathogenic role NMO. However, where how cell response altered remains controversial. In this study, we comprehensively analyzed with high-parameter flow cytometry immune populations CSF samples...
A small-molecule fluorescent probe specific for human pluripotent stem cells would serve as a useful tool basic cell biology research and therapy. Screening of chemical libraries with induced (iPSCs) subsequent evaluation hit molecules identified compound (Kyoto 1 [KP-1]) that selectively labels cells. Our analyses indicated the selectivity results primarily from distinct expression pattern ABC transporters in transporter KP-1. Expression ABCB1 (MDR1) ABCG2 (BCRP), both which cause efflux...
Alexander disease is a fatal neurological illness characterized by white-matter degeneration and formation of Rosenthal fibers, which contain glial fibrillary acidic protein as astrocytic inclusion. mainly caused gene mutation encoding protein, although the underlying pathomechanism remains unclear. We established induced pluripotent stem cells from patients, differentiated into astrocytes. patient astrocytes exhibited fiber-like structures, key pathology, increased inflammatory cytokine...
Mutations in the valosin-containing protein (VCP) gene were first found to cause inclusion- body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD). VCP later reported occur familial amyotrophic lateral sclerosis (ALS). But role of neurodegenerative processes that ALS remains unknown. The purpose present study was elucidate neurodegeneration seen sporadic mutant ALS. Immunohistochemistry demonstrated frequency distinct VCP-positive nuclei spinal motor neurons...
Dopamine (DA) is a neurotransmitter in the brain, playing central role several disease conditions, including tetrahydrobiopterin (BH4) metabolism disorders and Parkinson's (PD). BH4 present variety of clinical manifestations motor disturbance via altered DA metabolism, since cofactor for tyrosine hydroxylase (TH), rate-limiting enzyme synthesis. Genetically, are, an autosomal recessive pattern, caused by variant genes encoding enzymes synthesis or recycling, 6-pyruvoyltetrahydropterin...