240th ENMC workshop: The involvement of skeletal muscle stem cells in the pathology of muscular dystrophies 25 – 27 January 2019, Hoofddorp, The Netherlands

Twenty six participants representing patients, funding agencies and basic and clinical scientists involved in research into skeletal muscle stem cells and muscular dystrophies from France, Germany, Italy, India, UK, Australia, Spain, USA, The Netherlands and Switzerland met in Hoofddorp, The Netherlands on 25 – 27 January 2019. The meeting was held under the auspices of the ENMC and ENMC main sponsors, with the additional support of Muscular Dystrophy UK.
Source: Neuromuscular Disorders - Category: Neurology Authors: Tags: Workshop report Source Type: research

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Publication date: Available online 25 July 2019Source: Stem Cell ReportsAuthor(s): Takahiko Sato, Koki Higashioka, Hidetoshi Sakurai, Takuya Yamamoto, Naoki Goshima, Morio Ueno, Chie SotozonoSummaryThe use of adult skeletal muscle stem cells (MuSCs) for cell therapy has been attempted for decades, but still encounters considerable difficulties. MuSCs derived from human induced pluripotent stem cells (hiPSCs) are promising candidates for stem cell therapy to treat Duchenne muscular dystrophy (DMD). Here we report that four transcription factors, HEYL, KLF4, MYOD, and PAX3, selected by comprehensive screening of different Mu...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
AUF1 promotes rapid decay of mRNAs containing 3′ untranslated region (3′UTR) AU-rich elements (AREs). AUF1 depletion in mice accelerates muscle loss and causes limb girdle muscular dystrophy. Here, we demonstrate that the selective, targeted degradation by AUF1 of key muscle stem cell fate-determining checkpoint mRNAs regulates each stage of muscle...
Source: Proceedings of the National Academy of Sciences - Category: Science Authors: Tags: Biological Sciences Source Type: research
AbstractRotator cuff tears are common musculoskeletal injuries that can cause significant pain and disability. While the clinical results of rotator cuff repair can be good, failure of tendon healing remains a significant problem. Molecular mechanisms underlying structural failure following surgical repair remain unclear. Histologically, enhanced inflammation, disorganization of the collagen fibers, calcification, apoptosis and tissue necrosis affect the normal healing process. Mesenchymal stem cells (MSCs) have the ability to provide improved healing following rotator cuff repair via the release of mediators from secreted...
Source: Journal of Bone and Mineral Metabolism - Category: Orthopaedics Source Type: research
Conclusion and Perspectives The IL-6/JAK/STAT signaling cascade plays a dominant role in skeletal muscle pathophysiology. IL-6 autocrine, paracrine, and endocrine functions assign to its downstream effectors pivotal importance in skeletal muscle-wasting-associated diseases and other multiple system diseases where muscle acts in communication with other organs. Targeting the components of the JAK/STAT pathway recently emerged as a strategic approach for the treatment of inflammatory diseases and human cancer. This review highlights the opposite outcomes on muscle biology caused by the amount of local and systemic release ...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
In this study, we report the age-associated differences between fetal MSC (fMSC) populations and MSCs isolated from elderly donors with respect to their transcriptomes. We successfully reprogrammed fMSCs (55 days post conception) and adult MSC (aMSC; 60-74 years) to iPSCs and, subsequently, generated the corresponding iMSCs. In addition, iMSCs were also derived from ESCs. The iMSCs were similar although not identical to primary MSCs. We unraveled a putative rejuvenation and aging gene expression signature. We show that iMSCs irrespective of donor age and cell type re-acquired a similar secretome to that of th...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Reena Goswami1, Gayatri Subramanian2, Liliya Silayeva1, Isabelle Newkirk1, Deborah Doctor1, Karan Chawla2, Saurabh Chattopadhyay2, Dhyan Chandra3, Nageswararao Chilukuri1 and Venkaiah Betapudi1,4* 1Neuroscience Branch, Research Division, United States Army Medical Research Institute of Chemical Defense, Aberdeen, MD, United States 2Department of Medical Microbiology and Immunology, University of Toledo College of Medicine and Life Sciences, Toledo, OH, United States 3Roswell Park Comprehensive Cancer Center, Buffalo, NY, United States 4Department of Physiology and Biophysics, Case Western Reserve University, Clev...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Expression of the STAT3 gene influences a number of vital cellular processes, such as mitochondrial activity, cellular differentiation, and cellular proliferation. Researchers have investigated its activity in the context of spurring greater regenerative activity in heart muscles, for example. Arguably this is a good example of a regulatory gene that is involved in too many processes to make it a good target for therapeutics, however. More specific, lower-level mechanisms for specific desired goals would be helpful. That requires slow and costly investigative work, however, picking apart the relationships between proteins ...
Source: Fight Aging! - Category: Research Authors: Tags: Daily News Source Type: blogs
(Sanford Burnham Prebys Medical Discovery Institute) Scientists from Sanford Burnham Prebys have uncovered a molecular signaling pathway involving Stat3 and Fam3a proteins that regulates how muscle stem cells decide whether to self-renew or differentiate -- an insight that could lead to muscle-boosting therapeutics for muscular dystrophies or age-related muscle decline. The study was published in Nature Communications.
Source: EurekAlert! - Social and Behavioral Science - Category: International Medicine & Public Health Source Type: news
ConclusionRb1 and Rb2 upregulate myotube growth and myogenic differentiation through activating Akt/mammalian target of rapamycin signaling and inducing myogenic conversion of fibroblasts. Thus, our first finding indicates that Rb1 and Rb2 have strong potential as a helpful remedy to prevent and treat muscle atrophy, such as age-related muscular dystrophy.
Source: Journal of Ginseng Research - Category: Complementary Medicine Source Type: research
Contributor : Anthony M GacitaSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensMyotonic dystrophy (DM) is the most common autosomal dominant muscular dystrophy and encompasses both skeletal muscle and cardiac complications. Myotonic dystrophy is nucleotide repeat expansion disorder in which type 1 (DM1) is due to a trinucleotide repeat expansion on chromosome 19 and type 2 (DM2) arises from a tetranucleotide repeat expansion on chromosome 3. Developing representative models of myotonic dystrophy in animals has been challenging due to instability of nucleotide repeat expansions, especia...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
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