A5031033434- Muscle Physiology and Disorders
- Tissue Engineering and Regenerative Medicine
- Mesenchymal stem cell research
- Virus-based gene therapy research
- Adipose Tissue and Metabolism
- Exercise and Physiological Responses
- Pluripotent Stem Cells Research
- Genetic Neurodegenerative Diseases
- Silk-based biomaterials and applications
- RNA Interference and Gene Delivery
- Neurogenetic and Muscular Disorders Research
- Cardiomyopathy and Myosin Studies
- Muscle metabolism and nutrition
- Cell Adhesion Molecules Research
- Mitochondrial Function and Pathology
- Bone and Dental Protein Studies
- Biomedical Ethics and Regulation
- Advanced Sensor and Energy Harvesting Materials
- Spaceflight effects on biology
- Genetics, Aging, and Longevity in Model Organisms
- CAR-T cell therapy research
- Nitric Oxide and Endothelin Effects
- Knee injuries and reconstruction techniques
- Cellular Mechanics and Interactions
- Nuclear Structure and Function
National Center of Neurology and Psychiatry
2012-2023
National College Of Nursing
2019
Suzuki (Japan)
2017
National Neuroscience Institute
2012-2015
Tokyo Institute of Technology
2011
Accumulation of adipocytes and collagen type-I-producing cells (fibrosis) is observed in muscular dystrophies. The origin these had been largely unknown, but recently we identified mesenchymal progenitors positive for platelet-derived growth factor receptor alpha (PDGFRα) as the skeletal muscle. However, muscle fibrosis remains unknown. In this study, clonal analyses show that PDGFRα+ also differentiate into cells. fact, accumulated fibrotic areas diaphragm mdx mouse, a model Duchenne...
Abstract Skeletal muscle satellite cells play key roles in postnatal growth and regeneration. To study molecular regulation of cells, we directly prepared from 8- to 12-week-old C57BL/6 mice performed genome-wide gene expression analysis. Compared with activated/cycling 507 genes were highly upregulated quiescent cells. These included negative regulators cell cycle myogenic inhibitors. Gene set enrichment analysis revealed that preferentially express the involved cell-cell adhesion, growth,...
Forkhead box O (Foxo) transcription factors induce muscle atrophy by upregulating the muscle-specific E3 ubiquitin ligases MuRF-1 and atrogin-1/MAFbx, but other than Akt, upstream regulators of Foxos during are largely unknown. To examine involvement dystrophin glycoprotein complex (DGC) in regulation Foxo activities atrophy, we analyzed expression DGC members tail suspension, a model unloading-induced atrophy. Among several members, only neuronal NOS (nNOS) quickly dislocated from...
Schwann cells form basal laminae (BLs) containing laminin-2 (Ln-2; heterotrimer α2β1γ1) and Ln-8 (α4β1γ1). Loss of Ln-2 in humans mice carrying α2-chain mutations prevents developing from fully defasciculating axons, resulting partial amyelination. The principal pathogenic mechanism is thought to derive structural defects cell BLs, which scaffolds. However, we found loss caused amyelination without affecting BL structure or levels. Combined Ln-2/Ln-8 deficiency nearly complete amyelination,...
Satellite cells, which are skeletal muscle stem divide to provide new myonuclei growing fibers during postnatal development, and then maintained in an undifferentiated quiescent state adult muscle. This is considered be essential for the maintenance of satellite but their molecular regulation unknown. We show that Hesr1 (Hey1) Hesr3 (Heyl) (which known Notch target genes) expressed simultaneously only cells. In single-knockout mice, no obvious abnormalities cells or regenerative potentials...
Skeletal muscle contains two distinct stem/progenitor populations. One is the satellite cell, which acts as a stem and other mesenchymal progenitor, contributes to pathogeneses such fat infiltration fibrosis. Detailed accurate characterization of these progenitors in humans remains elusive. Here, we performed comprehensive cell-surface protein profiling progenitor populations residing human skeletal identified three previously unrecognized markers: CD82 CD318 for cells CD201 progenitors....
Calcitonin receptor (Calcr) is expressed in adult muscle stem cells (muscle satellite [MuSCs]). To elucidate the role of Calcr, we conditionally depleted Calcr from MuSCs and found that impaired regeneration after injury correlated with decreased number Calcr-conditional knockout (cKO) mice. signaling maintained MuSC dormancy via cAMP-PKA pathway but had no impact on myogenic differentiation an undifferentiated state. The abnormal quiescent state Calcr-cKO mice resulted a reduction pool by...
The myogenic potential of bone marrow and fetal liver cells was examined using donor from green fluorescent protein (GFP)-gene transgenic mice transferred into chimeric mice. Lethally irradiated X-chromosome-linked muscular dystrophy (mdx) receiving the exhibited significant numbers fluorescence+ dystrophin+ muscle fibres. In order to compare generating capacity with in neonatal chimeras,these two cell types were injected busulfantreated normal or mdx mice, generation chimeras examined....
Satellite cells are muscle stem that have important roles in postnatal growth and adult regeneration. Although fast- slow-dividing populations activated satellite been observed, the functional differences between them remain unclear. Here we elucidated relationship proliferation behaviour cell function. To assess frequency of division, isolated from mouse EDL were labelled with fluorescent dye PKH26, stimulated to proliferate then sorted by FACS. The vast majority PKH26(low) fast-dividing...
Abstract The laminin family of extracellular matrix proteins are expressed broadly during embryonic brain development, but enriched at ventricular and pial surfaces where laminins mediate radial glial attachment corticogenesis. In the adult brain, however, distribution is restricted, yet found within vascular basal lamina associated fractones zone (VZ)‐subventricular (SVZ) stem cell niche, regulate neural progenitor proliferation. It remains unknown, if wave oligodendrogenesis that occurs in...
Duchenne muscular dystrophy (DMD) is a lethal disorder of skeletal muscle caused by mutations in the dystrophin gene. Adeno-associated virus (AAV) vector-mediated gene therapy promising approach to disease. Although rod-truncated microdystrophin has been proven ameliorate dystrophic phenotypes, level expression required for effective an AAV vector not determined yet. Here, we constructed recombinant type 2 vector, AAV2-MCKDeltaCS1, expressing (DeltaCS1) under control muscle-specific MCK...
Satellite cells are myogenic stem responsible for the postnatal regeneration of skeletal muscle. Here we report successful in vitro induction Pax7-positive satellite-like from mouse embryonic (mES) cells. Embryoid bodies were generated mES and cultured on Matrigel-coated dishes with Dulbecco's modified Eagle medium containing fetal bovine serum horse serum. enriched by fluorescence-activated cell sorting using a novel anti-satellite antibody, SM/C-2.6. SM/C-2.6-positive efficiently...
Duchenne muscular dystrophy (DMD) is a lethal muscle disorder caused by mutations in the dystrophin gene. Transplantation of autologous myogenic cells genetically corrected ex vivo possible treatment for this disorder. In order to test regenerative efficiency freshly isolated satellite cells, we purified quiescent from limb muscles 8–12-week-old green fluorescent protein-transgenic (GFP-Tg) mice using SM/C-2.6 (a recently developed monoclonal antibody) and flow cytometry. Freshly were shown...
Hypoglycosylation and reduced laminin-binding activity of α-dystroglycan are common characteristics dystroglycanopathy, which is a group congenital limb-girdle muscular dystrophies. Fukuyama-type dystrophy (FCMD), caused by mutation in the fukutin gene, severe form dystroglycanopathy. A retrotransposal insertion seen almost all cases FCMD. To better understand molecular pathogenesis dystroglycanopathies to explore therapeutic strategies, we generated knock-in mice carrying mouse ortholog....