• When overexpressed in the murine myoblast cell line C2C12 or in primary myoblasts, C/EBPβ reduced MyoD and other myogenic protein levels during differentiation, in addition to reducing myoblast fusion. (biomedcentral.com)
  • Transplantation of myogenic stem cells possesses great potential for long-term repair of dystrophic muscle. (biomedcentral.com)
  • Embryonic myogenic progenitor cells express CXCR4, G-protein coupled cell surface receptor, and migrate towards regions of SDF-1 expression during limb muscle development, suggesting that CXCR4/SDF-1 plays a role in muscle cell homing. (biomedcentral.com)
  • Pluripotent embryonic stem cells (ESCs) have the unique ability to differentiate into every cell type and to self-renew. (biomedcentral.com)
  • Deletion of HP1β, but not HP1α, in ESCs provokes a loss of the morphological and proliferative characteristics of embryonic pluripotent cells, reduces expression of pluripotency factors and causes aberrant differentiation. (biomedcentral.com)
  • Embryonic stem cells (ESCs), derived from the blastocyst-stage embryo, are capable of generating all cell types of the mammalian body (pluripotency) and of maintaining the capacity for indefinite self-renewal without compromising their genomic integrity. (biomedcentral.com)
  • They are integral regulators of essential cell signaling pathways affecting cell growth, proliferation, differentiation, and migration. (wikipedia.org)
  • In-vitro studies with isolated SA progenitor cells had suggested that chromaffin cell differentiation depends crucially on glucocorticoids provided by adrenal cortical cells. (silverchair.com)
  • The transcription factor CCAAT/enhancer binding protein beta (C/EBPβ) is expressed in Pax7 + satellite cells of healthy muscle and is downregulated during myoblast differentiation. (biomedcentral.com)
  • However, in differentiated cells, loss of HP1β has the opposite effect, perturbing maintenance of the differentiation state and facilitating reprogramming to an induced pluripotent state. (biomedcentral.com)
  • We demonstrate an unexpected duality in the role of HP1β: it is essential in ESCs for maintaining pluripotency, while it is required for proper differentiation in differentiated cells. (biomedcentral.com)
  • This unique duality makes them an attractive system for potential regenerative medicine and cell therapies, but also for differentiation studies in vitro and for modeling diseases. (biomedcentral.com)
  • Myofiber-specific ablation of IRE1α or XBP1 in mice diminishes skeletal muscle regeneration that is accompanied with reduced number of satellite cells. (elifesciences.org)
  • Finally, targeted ablation of IRE1α also reduces Notch signaling, abundance of satellite cells, and skeletal muscle regeneration in the mdx mice, a model of Duchenne muscular dystrophy. (elifesciences.org)
  • Collectively, our experiments suggest that the IRE1α-mediated signaling promotes muscle regeneration through augmenting the proliferation of satellite cells in a cell non-autonomous manner. (elifesciences.org)
  • However, diprotin A, a positive modulator of CXCR4-SDF-1 binding, significantly enhanced engraftment and stimulated sustained proliferation of donor cells in vivo . (biomedcentral.com)
  • Diprotin A stimulation of CXCR4, however, significantly increased the number of canine dystrophin-positive muscle fibers and canine-derived satellite cells by enhancing donor cell proliferation. (biomedcentral.com)
  • In murine-to-murine transplantation experiments, CXCR4 expression marks a population of adult murine satellite cells with robust engraftment potential in mdx mice, and CXCR4-positive murine muscle-derived SP cells home more effectively to dystrophic muscle after intra-arterial delivery in mdx 5cv mice. (biomedcentral.com)
  • Skeletal muscle, the most abundant tissue of the body, has remarkable regenerative capacity mainly due to its resident muscle stem cells, also known as satellite cells. (elifesciences.org)
  • Ex vivo cultures of myofiber explants demonstrate that ablation of IRE1α reduces the proliferative capacity of myofiber-associated satellite cells. (elifesciences.org)
  • In vitro and in vivo studies of dysferlinopathy have identified metabolic effects are often distinct or at odds with observations from other types of muscular dystrophy. (jain-foundation.org)
  • There his work focused on transcriptional control of hematopoietic stem cell maturation and cell fate. (ubc.ca)
  • In addition, he identified a number of novel hematopoietic stem cell surface proteins and began analyzing their function. (ubc.ca)
  • His laboratory has followed two primary interests: 1) the transcription factor networks that regulate fate determination in various cells that make blood, and 2) the cell surface proteins expressed by hematopoietic stem cells that and allow them to communicate with their microenvironment. (ubc.ca)
  • In this regard, his lab has identified a novel family of hematopoietic cell surface proteins, called the CD34 family, and shown that these are essential for a number of developmentally important processes. (ubc.ca)
  • We used a clinically acceptable regimen of hematopoietic stem cell transplantation to establish mixed donor/host blood cell chimerism and immune tolerance in a canine model of Duchenne muscular dystrophy ( cxmd ) [ 9 ]. (biomedcentral.com)
  • Therefore, we sought to translate these results to a clinically relevant canine-to-canine allogeneic transplant model for Duchenne muscular dystrophy (DMD) and determine if CXCR4 is important for donor cell engraftment. (biomedcentral.com)
  • Intramuscular injection of donor muscle-derived cells into chimeric cxmd recipients restored dystrophin expression for at least 24 weeks in the absence of post-transplant immunosuppression, indicating that cell transplantation may be a viable therapeutic option for muscular dystrophy. (biomedcentral.com)
  • Metabolic changes in skeletal muscle are a hallmark of several types of muscular dystrophy. (jain-foundation.org)
  • Postnatal growth and repair of skeletal muscle relies upon a population of quiescent muscle precursor cells, called satellite cells that can be activated to proliferate and differentiate into new myofibers, as well as self-renew to replenish the satellite cell population. (biomedcentral.com)
  • However, analysis of mice lacking the glucocorticoid receptor gene had revealed that adrenal chromaffin cells develop mostly normally in these mice. (silverchair.com)
  • After a second round of injury, muscle regeneration is impaired in C/EBPβ conditional knockout mice compared to wild-type control mice. (biomedcentral.com)
  • Indeed, CXCR4-positive muscle-derived side population (SP) cells home more effectively to dystrophic muscle after intra-arterial delivery in mdx 5cv mice [ 10 ]. (biomedcentral.com)
  • In this study, we used a canine-to-murine xenotransplantation model to quantitatively compare canine muscle cell engraftment, and test the most effective cell population and modulating factor in a canine model of DMD using allogeneic transplantation experiments. (biomedcentral.com)
  • Furthermore, the canine-to-murine xenotransplantation model accurately predicted results in canine-to-canine muscle cell transplantation. (biomedcentral.com)
  • Yet, it remains unclear from murine transplantation experiments which cell population most effectively engrafts into diseased skeletal muscle. (biomedcentral.com)
  • After a single muscle injury, C/EBPβ-deficient satellite cells fail to self-renew resulting in a reduction of satellite cells available for future rounds of regeneration. (biomedcentral.com)
  • He has delineated the function of these molecules in diverse set of biological processes including: 1) gut and kidney formation, 2) vascular permeability, 3) mucosal inflammatory disease, 4) stem cell homing and migration, and 5) epithelial tumor progression. (ubc.ca)
  • We demonstrate here that loss of the primary cilium on postnatal biliary epithelial cells (via the deletion of the cilia gene Wdr35) drives ongoing pathological remodeling of the biliary tree, resulting in progressive cyst formation and growth. (bvsalud.org)
  • Using genetic tools to conditionally abrogate C/EBPβ expression in Pax7 + cells, we examined the role of C/EBPβ in self-renewal of satellite cells during muscle regeneration. (biomedcentral.com)
  • These findings demonstrate that C/EBPβ is a novel regulator of satellite cell self-renewal during muscle regeneration acting at least in part through Notch2. (biomedcentral.com)
  • The findings shed new light on mechanisms of regeneration of healthy myofibers after severe tissue injury and suggest interplay between these fibers and muscle satellite cells though mechanisms remain to be elucidated. (elifesciences.org)
  • He is a member of the Stem Cell Network Centre of Excellence (past member of the Stem Cell Policy Committee and Research Management Committee and current Sub-chair of the Training and Education Committee), and a member of the AllerGen Network Centre of Excellence (Research Management Committee and Co-Chair of the Biomarkers Program). (ubc.ca)
  • Therefore, these results establish the efficacy of diprotin A in stimulating muscle cell engraftment, and highlight the pre-clinical utility of a xenotransplantation model in assessing the relative efficacy of muscle stem cell populations. (biomedcentral.com)
  • HS chains are heterogeneous structures that differ in specific and conditional cell contexts. (wikipedia.org)
  • Focusing on a set of driver mutations that interact with KRAS to initiate aggressive, sarcomatoid-type ICC revealed that tumor growth relies on Wnt and PI3K signaling. (bvsalud.org)
  • 93. Nonetheless, you can find number of reports to the validity in the quick form model, none in Persian language, as well as much less study to assess the psychometric properties in the quick type versus the extended kind. (thrombininhibitors.com)
  • There he worked with Dr. Max D. Cooper (Howard Hughes Medical Institute, National Academy of Sciences) and his research focused on cell surface proteins expressed by preB cells that regulate B cell maturation and homing. (ubc.ca)
  • Through gene knockout studies he has shown that these molecules act as a type of molecular "Teflon" to make cells more mobile and invasive and also facilitate chemotaxis. (ubc.ca)
  • Discussion In most from the previously published scientific studies on HRQoL in PD, the lengthy type 39 item questionnaire is validated in numerous languages. (thrombininhibitors.com)
  • These cells are located beneath the basal lamina of myofiber in a dormant state. (elifesciences.org)
  • Panobinostat therapy significantly repressed mRNA for DNMT1 and DNMT3a in the two cell lines while no modifications had been observed in DNMT3b levels. (thrombininhibitors.com)
  • Before the cloning and characterization of Sulf1 and Sulf2, HS composition was thought to be unchanging after localization to the cell surface. (wikipedia.org)
  • In contrast to lysosomal active sulfatases, QSulf1 localizes exclusively to the cell surface by interacting hydrophilically with a non-heparan sulfate outer membrane component, and is enzymatically active at a neutral pH. (wikipedia.org)
  • Importantly, proper glycosylation is necessary to localize to the cell surface, possibly to bind HS moieties, and was required for enzymatic activity. (wikipedia.org)
  • We used the xenotransplant model to show that CXCR4 expression on canine donor muscle cells is also important for cell engraftment, but FACS sorting for CXCR4-positive cells decreased their engraftment efficiency. (biomedcentral.com)
  • Our study indicates that primary ciliary dyskinesia (PCD), caused by mutations in dynein assembly factors disrupting cytoplasmic pre-assembly of axonemal dynein motors, should be considered a cell-type specific protein-misfolding disease. (bvsalud.org)
  • Even to the lengthy type version, our examination showed that although each of the PDQ 39 objects correlate very well with their scale distinct total score, dimensions this kind of as social support. (thrombininhibitors.com)
  • We show that pharmacological inhibition of FKBP8 phenocopies dynein motor instability associated with the loss of ZMYND10 in airway cells and that human disease-causing variants of ZMYND10 disrupt its ability to act as an FKBP8-HSP90 co-chaperone. (bvsalud.org)
  • We show that CXCR4 expressing cells are important for donor muscle cell engraftment, yet FACS sorted CXCR4-positive cells display decreased engraftment efficiency. (biomedcentral.com)
  • Together, these data suggest that CXCR4 plays an important role in donor cell engraftment. (biomedcentral.com)
  • Heparan sulfate proteoglycans (HSPGs) act as co-receptors for numerous heparin-binding growth factors and cytokines and are involved in cell signaling. (wikipedia.org)
  • The diversification of neural-crest-derived sympathoadrenal (SA) progenitor cells into sympathetic neurons and neuroendocrine adrenal chromaffin cells was thought to be largely understood. (silverchair.com)
  • The function of HSPGs extends beyond providing an extracellular matrix (ECM) structure and scaffold for cells. (wikipedia.org)