Download or read book Modeling of Skeletal Muscle Development and Disease Using Human Stem Cells written by Saowanee Jiwlawat and published by . This book was released on 2017 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Stem cell technology provides a new critical cell resource for basic and translational research, and their unique properties of indefinite proliferation and multi-cell lineage differentiation greatly benefit cellular modeling and therapy. By simulating small molecule signaling during embryonic myogenesis, myogenic progenitors can be successfully generated from human induced-pluripotent stem cells (iPSCs). The main objective of this thesis is to prepare skeletal muscles using human pluripotent stem cells, and to explore their ability as [in vitro] models to study skeletal muscle development and disorders. First, we tested the hypothesis of how differentiation duration, culture surface coatings, and nutrient supplements affect muscle development (especially myotube and sarcomere formation). Myogenic progenitors were prepared from human iPSCs using free-floating spherical culture and then differentiated into myotubes. The ultrastructure of iPSC-derived myotubes revealed well-organized sarcomere structures after a long differentiation period over 6 weeks. Next, we used patient-derived iPSCs with Pompe disease and evaluated the ability of these skeletal myocytes to act as [in vitro] disease models for studying disease mechanisms and testing drug candidates. Pompe iPSC-derived myotubes exhibited specific disease phenotypes, including reduction of acid alpha-glucosidase enzyme activity and abnormal accumulation of glycogen in enlarged lysosomes. We also attempted to repair cellular pathology in Pompe iPSC-derived myotubes by using specific compounds that could potentially modulate transcription factor EB signaling or glycogen metabolism. Lastly, we applied bioengineering approaches to improve alignment, sarcomere formation, and spontaneous contractions of human iPSC-derived myotubes. The micropatterned substrate with controlled stiffness significantly promoted fusion and differentiation of iPSC-derived myocytes, leading to the formation of well-aligned and multi-nucleated myotubes. Notably, these aligned myotubes exhibited spontaneous contractions specifically along the long axis of the pattern. On the bioengineered culture platform, bundle-like myotubes were created from patient-derived iPSCs with Pompe disease, which showed specific pathology such as abnormal accumulations of enlarged lysosomes. Using healthy- and patient-derived iPSC lines with a neuromuscular disorder, we successfully created matured skeletal myotubes in culture. These myotubes morphologically and functionally resemble myotubes found in adult skeletal muscles. This matured muscle model would be enormously valuable to study skeletal muscle development, disease mechanisms, and would aid in pharmacological studies.
Download or read book Skeletal Muscle Stem Cells written by Atsushi Asakura and published by Springer Nature. This book was released on 2023-03-30 with total page 472 pages. Available in PDF, EPUB and Kindle. Book excerpt: This volume looks at the latest technologies and methods--combined with new genetic tools available in animal models--used in this constantly evolving field. The chapters in this book are organized into three sections: Section one covers muscle stem cells and progenitor cells; Section Two discusses animal models for muscle stem cells and regeneration; and Section Three explores bioinformatics and imaging analysis for muscle stem cells. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Cutting-edge and comprehensive, Skeletal Muscle Stem Cells and Regeneration: Methods and Protocols is a valuable tool for all researchers looking to expand their knowledge on skeletal muscle growth, repair, degeneration, aging, and regenerative medicine.
Download or read book Muscle Regeneration written by Alexander Mauro and published by Raven Press (ID). This book was released on 1979 with total page 582 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Engineered Skeletal Muscle from Human Pluripotent Stem Cells to Model Muscle Disease and Regeneration written by Mina Shahriyari and published by . This book was released on 2021 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Skeletal muscle disease modeling offers the unique opportunity to investigate devastating muscle diseases like Duchenne Muscle Dystrophy in vitro but requires advanced three-dimensional (3D) model systems reflecting the characteristics of human muscle in vivo. The aim of this study was to generate engineered models of skeletal muscle from human pluripotent stem cells (hPSCs) with physiological properties by recapitulating specific stages of muscle development. To allow for robust skeletal muscle tissue engineering first a directed differentiation protocol was established in 2D culture under...
Download or read book Establishing an in Vitro Model of Skeletal Muscle Development and Aging Using Human Stem Cells written by Sin-Ruow Tey and published by . This book was released on 2022 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Stem cells have the unique properties of indefinite proliferation and multi-cell lineage differentiation, thereby providing a new unlimited cell resource for cellular modeling in basic and translational research. Myogenic progenitors can be successfully generated from human pluripotent stem cells (hPSCs) without genetic modifications by simulating small molecule signaling during embryonic myogenesis. The main objective of this thesis is to explore the utilizations of hPSC-derived myogenic progenitors as in vitro models to study skeletal muscle development and aging under natural and pathological conditions. First, we demonstrated that cell surface proteins can be used as markers to enrich and characterize hPSC-derived myogenic progenitors, as well as to identify new pathways through their involvements in myogenesis. We discovered that myotube formation was elevated in differentiated cultures of CD29+, CD56+, CD271+, and CD15- fractions, while myotube fusion and maturation were more prominent in the differentiated cultures from CD9+ and CD146+ fractions. We showed that CD271 expression is essential for terminal differentiation of hPSC-derived myogenic progenitors. Next, we developed in vitro culture systems of hPSC-derived myogenic progenitors to study age-related muscle degeneration. We found that supplementing hPSC-derived myogenic progenitors with aged rat sera impaired their self-renewal capacity and myotube formation potential. We identified indications of aberrant cell cycle events as the contributing factors to such aging phenotypes. Then, we replicated our work using supplementation of plasma from aged human donors. Our results confirmed that circulating factors in aged systemic biokinetics exerted inhibitory effects on viability and proliferation of hPSC-derived myogenic progenitors. We validated that treatment with plasma from healthy elders and patients of Alzheimer's disease during terminal differentiation led to abnormal morphological changes in myotubes: myotubes were enlarged and misshapen, and myonuclei were centrally aggregated. These observations again pointed towards disruption in cell cycle regulation. Lastly, we attempted to make new connections between age-related dysregulation of the balance of hypothalamus-pituitary-gonadal (HPG) axis, cell cycle aberrancy and muscle degeneration. We treated hPSC-derived myogenic progenitors with bioidentical HPG hormones at the concentrations reported in post-menopausal women to mimic endocrine dyscrasia. Our preliminary data suggests that age-specific imbalance of HPG hormones reduced expansion of hPSC-derived myogenic progenitors and contributed towards nuclear aggregation during myotube formation. Together, we successfully illustrated the feasibility of hPSC-derived culture systems as a powerful platform to investigate clinically relevant mechanisms of skeletal muscle development and aging.
Download or read book Role of Stem Cells in Skeletal Muscle Development Regeneration Repair Aging and Disease written by Pura Muñoz-Cánoves and published by Frontiers Media SA. This book was released on 2016-06-29 with total page 222 pages. Available in PDF, EPUB and Kindle. Book excerpt: Adult stem cells are responsible for tissue regeneration and repair throughout life. Their quiescence or activation are tightly regulated by common signalling pathways that often recapitulate those happening during embryonic development, and thus it is important to understand their regulation not only in postnatal life, but also during foetal development. In this regard, skeletal muscle is an interesting tissue since it accounts for a large percentage of body mass (about 40%), it is highly amenable to intervention through exercise and it is also key in metabolic and physiological changes underlying frailty susceptibility in the elderly. While muscle-resident satellite cells are responsible for all myogenic activity in physiological conditions and become senescent in old age, other progenitor cells such as mesoangioblasts do seem to contribute to muscle regeneration and repair after tissue damage. Similarly, fibro-adipogenic precursor cells seem to be key in the aberrant response that fills up the space left from atrophied muscle mass and which ends up with a dysfunctional muscle having vast areas of fatty infiltration and fibrosis. The complex interplay between these stem/progenitor cell types and their niches in normal and pathological conditions throughout life are the subjects of intense investigation. This eBook highlights recent developments on the role of stem cells in skeletal muscle function, both in prenatal and postnatal life, and their regulation by transcriptional, post-transcriptional and epigenetic mechanisms. Additionally, it includes articles on interventions associated with exercise, pathological changes in neuromuscular diseases, and stem cell aging.
Download or read book Muscle Cell and Tissue written by Kunihiro Sakuma and published by BoD – Books on Demand. This book was released on 2018-10-10 with total page 372 pages. Available in PDF, EPUB and Kindle. Book excerpt: In order to complete tissue regeneration, various cells (neuronal, skeletal and smooth) interact coordinately with each other. This book, Muscle Cell and Tissue - Current Status of Research Field, deals with current progress and perspectives in a variety of topics on the skeletal and smooth muscle, stem cells, regeneration, disease or therapeutics. Novel applications for cell and tissue engineering including cell therapy, tissue models and disease pathology modeling are introduced. This book also deals with the differentiation/de-differentiation process of vascular smooth muscle cells in health and disease. Furthermore, natural products to reverse metabolic syndromes are descriptively reviewed. These chapters can be interesting for graduate students, teachers, physicians, executives and researchers in the field of molecular biology and regenerative medicine.
Download or read book Muscle Stem Cells written by Eusebio Perdiguero and published by Springer Nature. This book was released on 2017-02-27 with total page 412 pages. Available in PDF, EPUB and Kindle. Book excerpt: This volume provides leading-edge protocols in the study of the molecular and cellular biology of muscle stem cells. Chapters detail current and updated methods for muscle stem cell isolation, culture, molecular analysis, cellular analysis, and reintroduction in vivo as well as protocols for studying myogenic stem cells in non-mammalian model systems. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Authoritative and cutting-edge, Muscle Stem Cells: Methods and Protocols aims to ensure successful results in the further study of this vital field.
Download or read book Roles of Skeletal Muscle in Organ Development written by Boris Kablar and published by Springer Nature. This book was released on 2023-11-13 with total page 166 pages. Available in PDF, EPUB and Kindle. Book excerpt: Muscle is the only tissue of the four basic types that make the body that can be completely ablated while allowing fetal survival. This book is a result of 25 years of research employing engineered mouse fetuses with no skeletal muscle, a model system that provides a unique opportunity to study body development holistically. A systematic anatomical analysis of such fetuses have shown that several anatomical locations are affected by the absence of the skeletal muscle. This book contains a summarized description of affected anatomical locations such as the alveolar lung epithelium, motor neurons and giant pyramidal cells in the CNS, cholinergic amacrine cells of the retina, and type I hair cells of the crista ampullaris. Several specific bioinformatics and systems biology interventions are also described. The book provides an update on skeletal muscle development, musculoskeletal developmental interactions, trophic relationships between the skeletal muscle and the motor neurons, mechanics of lung development, functional development of two special senses, eye and ear, and finally, skeletal muscle-related reasons for human fetal akinesia and its consequences. This volume in the Advances in Anatomy, Embryology and Cell Biology series stresses the need to think about the developing body and its organs in terms of their mutual interdependence, and to think about diseases, such as pulmonary hypoplasia, amyotrophic lateral sclerosis, or cleft palate, in terms of that interdependence. Directed to developmental biologists, neuroscientists, tissue engineers and health professionals, this book exposes the ideas of interorgan communication and interdependence in homeostasis and disease.
Download or read book Stem Cells in Modeling Human Genetic Diseases written by Mayana Zatz and published by Springer. This book was released on 2015-08-11 with total page 157 pages. Available in PDF, EPUB and Kindle. Book excerpt: While most stem cell books focus on basic aspects and/or cell therapy, this book emphasizes the relevance of stem cells obtained from patients, the so-called “patients in a petri dish” as tools to investigate human genetic diseases for which there are no available effective treatment. Chapters embrace several examples of the use of iPS cell technology, a recent Nobel Prize-winning scientific breakthrough, to obtain patient-specific pluripotent cells from which many types of specialized cells involved in a particular disease can be generated, including psychiatric and neurodegenerative disorders, muscular dystrophies, laminopathies, among others. The text is a current and timely resource for postgraduate students, scientists and clinicians, interested in applications of this rapidly developing field of research in disease modeling, drug development, and emerging issues that it brings to regenerative medicine.
Download or read book Making Skeletal Muscle from Human Pluripotent Stem Cells written by Ken'ichiro Nogami and published by . This book was released on 2018 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: Human pluripotent stem cells (hPSCs) proliferate in vitro for long periods without losing pluripotency and can be induced to differentiate into various cell types including skeletal muscle cells (SMCs). Human embryonic stem cells (hESCs) are generated from a preimplantation-stage embryo. Human-induced pluripotent stem cells (hiPSCs) are derived from somatic cells of both healthy donors and patients with muscle diseases of any age using reprogramming factors. Currently, there are two kinds of protocols to induce skeletal muscle from hPSCs. One type utilizes overexpression of a potent myogenic master regulator, MyoD, to directly induce skeletal muscle. Stepwise induction of skeletal muscle has also been reported by many research groups, but hiPSC-based cell therapy for muscular dystrophy is still experimental. On the other hand, hiPSCs derived from patients with muscle disease are widely used for disease modeling in vitro. Here, we review the recent literature on derivation of skeletal muscle from human pluripotent stem cells and discuss their application.
Download or read book Modeling Spinal Bulbar Muscular Atrophy in Vitro Using Human Skeletal Muscle Derived from Induced Pluripotent Stem Cells written by Garrett R. Wong and published by . This book was released on 2017 with total page 40 pages. Available in PDF, EPUB and Kindle. Book excerpt: Spinal bulbar muscular atrophy is a motor neuron disease caused by a poly-glutamine (polyQ) expansion in the first exon of the androgen receptor (AR) on the X chromosome. The expanded AR demonstrates a pathogenic protein causing autophagy dysregulation in neurons and skeletal muscle through alterations AR nuclear translocation. Until a few years ago, it was widely believed that the atrophy of the skeletal muscle was due to the loss of motor neurons, but recent studies supported the skeletal muscle's contribution in the disease. One of these studies involved the muscle specific knockdown of the mutant AR, leading to a complete rescue of mouse. Additionally, SBMA patients' skeletal muscle biopsies expressed signs of myopathy and changes in mitochondria morphology. With the field shifting towards examining skeletal muscle's role in SBMA, there is a need for an in vitro human skeletal muscle model of SBMA. Using skeletal muscle derived from human induced pluripotent stem cells (hiPSCs) from patients, this study hoped to determine if SBMA skeletal muscle exhibits a relevant phenotype of SBMA disease in a dish. hiPSCs were differentiated into skeletal muscles through over expression of the myogenic transcription factor (MyoD) and chromatin remodeling protein (BAF60C) followed by culturing the cells with horse serum. SBMA skeletal muscles with an expanded AR expressed an increase in toxicity characterized by increased mitochondria fragmentation and DNA damage via double stranded breaks. These results established a possible platform for in vitro SBMA disease modeling and drug screening.
Download or read book Research on Skeletal Muscle Diseases Using Pluripotent Stem Cells written by Lorena de Oñate and published by . This book was released on 2015 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: The generation of induced pluripotent stem cells (iPSCs), especially the generation of patient-derived pluripotent stem cells (PSCs) suitable for disease modelling in vitro, opens the door for the potential translation of stem-cell related studies into the clinic. Successful replacement, or augmentation, of the function of damaged cells by patient-derived differentiated stem cells would provide a novel cell-based therapy for skeletal muscle-related diseases. Since iPSCs resemble human embryonic stem cells (hESCs) in their ability to generate cells of the three germ layers, patient-specific iPSCs offer definitive solutions for the ethical and histo-incompatibility issues related to hESCs. Indeed human iPSC (hiPSC)-based autologous transplantation is heralded as the future of regenerative medicine. Interestingly, during the last years intense research has been published on disease-specific hiPSCs derivation and differentiation into relevant tissues/organs providing a unique scenario for modelling disease progression, to screen patient-specific drugs and enabling immunosupression-free cell replacement therapies. Here, we revise the most relevant findings in skeletal muscle differentiation using mouse and human PSCs. Finally and in an effort to bring iPSC technology to the daily routine of the laboratory, we provide two different protocols for the generation of patient-derived iPSCs.
Download or read book Muscle Homeostasis and Regeneration written by Antonio Musarò and published by MDPI. This book was released on 2020-11-20 with total page 500 pages. Available in PDF, EPUB and Kindle. Book excerpt: The book is a collection of original research and review articles addressing the intriguing field of the cellular and molecular players involved in muscle homeostasis and regeneration. One of the most ambitious aspirations of modern medical science is the possibility of regenerating any damaged part of the body, including skeletal muscle. This desire has prompted clinicians and researchers to search for innovative technologies aimed at replacing organs and tissues that are compromised. In this context, the papers, collected in this book, addressing a specific aspects of muscle homeostasis and regeneration under physiopathologic conditions, will help us to better understand the underlying mechanisms of muscle healing and will help to design more appropriate therapeutic approaches to improve muscle regeneration and to counteract muscle diseases.
Download or read book Myoblast Transfer Therapy written by Robert C. Griggs and published by Springer. This book was released on 2012-03-28 with total page 330 pages. Available in PDF, EPUB and Kindle. Book excerpt: I am pleased to introduce this volume on Myoblast Transfer Therapy on behalf of the Muscular Dystrophy Association and all of its Advisory Committees. The international conference which led to this volume brought together leading basic scientists and clinical investigators for the purpose of coordinating the development of this new field in the fight against muscular dystrophy. The Muscular Dystrophy Association is the nation's most rapidly growing voluntary health agency in terms of its programs of patient care, research, and professional and public education. Success is attributable to its National Chairman, Jerry Lewis, to its effective corporate membership, and to the many physicians and scientists who give their time freely to advise on policies, to review grant applications, and to participate in meetings such as this. I should like to acknowledge a large number of other individuals to whom we are indebted: the broad segment of the American public which continually and generously supports our spectrum of services. The Muscular Dystrophy Association, next year, should raise in excess of $115,000,000. These contributions are derived from more than 10 million American families. These families are not only pledging their money but expressing their hopes that we will find answers to the tragic problem of neuromuscular disease. We are confident that the fruits of this meeting will move the frontier of research forward toward that goal.
Download or read book Muscle Stem Cells written by and published by Elsevier. This book was released on 2024-05 with total page 486 pages. Available in PDF, EPUB and Kindle. Book excerpt: Muscle Stem Cells, Volume 158 in the Current Topics of Developmental Biology series, highlights new advances in the field, with this new volume presenting interesting chapters on topics surrounding Muscle stem cell dysfunction in rhabdomyosarcoma and muscular dystrophy, Model systems used to study MuSC function, MuSCs in the growth and maintenance of muscle, Molecular regulation of myocyte fusion, A self-made quiescent niche of muscle stem cells, Characterization of the muscle regenerative environment, Role of microenvironment on muscle stem cell function in health, adaptation, and disease, Vascular Niche for Muscle Stem Cells, Regulation of muscle stem cell polarity in health and disease, and more. Additional chatpers cover Circadian timing of satellite cell function and muscle regeneration, Muscle stem cell activity is regulated by translational control of gene expression, Biomechanical stress in modulating MuSC function, Cross talk between cell types in regenerating muscle, Effects of the immune system on muscle regeneration, Effects of diabetes on MuSC function, and other timely topics.
Download or read book Role of Stem Cells in Skeletal Muscle Development Regeneration Repair Aging and Disease written by and published by . This book was released on 2016 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Adult stem cells are responsible for tissue regeneration and repair throughout life. Their quiescence or activation are tightly regulated by common signalling pathways that often recapitulate those happening during embryonic development, and thus it is important to understand their regulation not only in postnatal life, but also during foetal development. In this regard, skeletal muscle is an interesting tissue since it accounts for a large percentage of body mass (about 40%), it is highly amenable to intervention through exercise and it is also key in metabolic and physiological changes underlying frailty susceptibility in the elderly. While muscle-resident satellite cells are responsible for all myogenic activity in physiological conditions and become senescent in old age, other progenitor cells such as mesoangioblasts do seem to contribute to muscle regeneration and repair after tissue damage. Similarly, fibro-adipogenic precursor cells seem to be key in the aberrant response that fills up the space left from atrophied muscle mass and which ends up with a dysfunctional muscle having vast areas of fatty infiltration and fibrosis. The complex interplay between these stem/progenitor cell types and their niches in normal and pathological conditions throughout life are the subjects of intense investigation. This eBook highlights recent developments on the role of stem cells in skeletal muscle function, both in prenatal and postnatal life, and their regulation by transcriptional, post-transcriptional and epigenetic mechanisms. Additionally, it includes articles on interventions associated with exercise, pathological changes in neuromuscular diseases, and stem cell aging.
