• US 20130022666 provides compositions comprising a lipid carrier and mitochondria as well as methods of delivering exogenous mitochondria to a cell and methods of treating or reversing progression of a disorder associated with mitochondrial dysfunction in a mammalian subject in need thereof. (justia.com)
  • Mitochondrial dysfunction has been proven to play a critical role in the pathogenesis of cardiovascular diseases. (frontiersin.org)
  • Maple syrup urine disease (MSUD) is rare autosomal recessive metabolic disorder caused by the dysfunction of the mitochondrial branched-chain 2-ketoacid dehydrogenase (BCKD) enzyme complex leading to massive accumulation of branched-chain amino acids and 2-keto acids. (stanford.edu)
  • Mitochondrial function or the ability to generate energy through OXPHOS (oxidative phosphorylation) is vital for cell homeostasis and its dysfunction has been linked to the pathogenesis of nearly all chronic diseases ( Pieczenik and Neustadt, 2007 ). (frontiersin.org)
  • Relevant to the studies presented in this manuscript, mitochondrial dysfunction is a key contributor to the perpetuation of metabolic inflammation caused by nutrient excess. (frontiersin.org)
  • Besides OXPHOS, mitochondrial dysfunction is also present in the examination of mitochondrial morphology, the balance of mitochondrial network formation, and fission and fusion. (frontiersin.org)
  • Apart from the indispensable role of the ovary in the overall hormonal milieu, ovarian dysfunction also affects many other organ systems and functions including sexuality, bones, the cardiovascular system, and neurocognitive function. (bvsalud.org)
  • Thirdly, the following experiments demonstrated that Cis treatment could induce mitochondrial dysfunction through overproducing superoxide in GCs and trigger lipid peroxidation leading to ferroptosis, first reported in chemotherapy-induced ovarian damage. (bvsalud.org)
  • Our study confirmed the chemotherapy-induced chaotic hormonal state and ovarian damage in preclinical and clinical examination and indicated that chemotherapeutic drugs initiated ferroptosis in ovarian cells through excessive ROS-induced lipid peroxidation and mitochondrial dysfunction, leading to ovarian cell death. (bvsalud.org)
  • Symptoms related to bone marrow dysfunction, such as anemia, leukopenia, or thrombocytopenia, are rare at presentation but can also be observed in the later stages of the disease. (medscape.com)
  • Mitochondrial dysfunction: roles in skeletal muscle atrophy. (stembook.org)
  • Thus, we propose that HNF-1links extracellular inflammatory signals to mitochondrial dysfunction during AKI partly PPARGC1A signaling. (bms-911543.com)
  • Results AKI Is normally Accompanied by HNF-1Inhibition and Mitochondrial Dysfunction So that they can better understand the function of HNF-1during AKI, we initial assessed its appearance aswell as the appearance of its focus on genes within a mouse style of sepsis-induced AKI. (bms-911543.com)
  • Preclinical models support the hypothesis that MSCs may have clinical efficacy in COPD. (bmj.com)
  • 2 MSCs were first described in the bone marrow where they constitute a small fraction of cells (0.001%-0.01%) that closely interact with haematopoietic cells to support haematopoiesis and skeletal homeostasis. (bmj.com)
  • 3 4 Since then, it has become evident that MSCs reside in many tissues, including mesenchymal tissues (bone, adipose tissue, connective tissue), umbilical cord and several organs including the liver, spleen and lung. (bmj.com)
  • In the present study, we examine the specific impacts of MSCs on mitochondrial morphology and function in host tissues. (frontiersin.org)
  • Employing in vitro cell culture of inherited mitochondrial disease and an in vivo animal experimental model of low-grade inflammation (high fat feeding), we show human-derived MSCs to alter mitochondrial function. (frontiersin.org)
  • Additionally, as a novel source of MSCs, menstrual blood-derived endometrial stem cells (MenSCs) have exhibited promising therapeutic effects in various diseases due to their comprehensive advantages, such as periodic and non-invasive sample collection, abundant sources, regular donation and autologous transplantation. (bvsalud.org)
  • Therefore, this review summarizes the efficacy of MSCs transplantation in improving chemotherapy-induced POI and analyzes the underlying mechanism, and further discusses the benefit and existing challenges in promoting the clinical application of MenSCs in chemotherapy-induced POI. (bvsalud.org)
  • This study aims to verify the safety, feasibility, and efficacy of MSCs treatment in canine idiopathic autoimmune inflammatory disorders of the CNS. (biomedcentral.com)
  • Autologous bone marrow MSCs (BMMSCs), isolated, cultured, and expanded, were administered by intrathecal (IT) injection in the cisterna magna intravenously (IV) and by intra-arterial (IA) injection in the right carotid artery. (biomedcentral.com)
  • In this study, mesenchymal stem cells isolated from the bone-marrow of mice (BM MSCs), were labeled with Hoechst after low (3 to 8) or high (40 to 50) numbers of passages and then transplanted intrastriatally into 5-week-old R6/2 mice, which carries the N-terminal fragment of the human HD gene (145 to 155 repeats) and rapidly develops symptoms analogous to the human form of the disease. (biomedcentral.com)
  • These beneficial effects are likely due to trophic support, as an increase in brain derived neurotrophic factor mRNA expression was observed in the striatum following transplantation of BM MSCs. (biomedcentral.com)
  • The results from this study demonstrate that BM MSCs hold significant therapeutic value for HD, and that the amount of time the cells are exposed to in vitro culture conditions can alter their efficacy. (biomedcentral.com)
  • The phenomenon of intercellular mitochondrial transfer has been discovered in the cardiovascular system. (frontiersin.org)
  • The intercellular mitochondrial transfer was reported for the first time by Spees and colleagues in 2006. (frontiersin.org)
  • However, harmful stimuli (such as ischemia-reperfusion, oxidative stress, and toxic chemicals) can change the direction and efficiency of intercellular mitochondrial transfer. (frontiersin.org)
  • Intercellular mitochondrial transfer occurs in different ways, including tunnelling nanotubes (TNTs), extracellular vesicles (EVs), and gap junction channels (GJCs). (biomedcentral.com)
  • According to research on intercellular mitochondrial transfer in physiological and pathological environments, mitochondrial transfer hold great potential for maintaining body homeostasis and regulating pathological processes. (biomedcentral.com)
  • In addition to intracellular mitochondrial movement, intercellular mitochondrial transfer between mammalian cells has recently been discovered. (biomedcentral.com)
  • Intercellular mitochondrial transfer can be regarded as an extension of intracellular mitochondrial movement or intercellular communication, which undoubtedly increases the mtDNA content of the recipient cells and restores the respiration and survival of the recipient cells. (biomedcentral.com)
  • Although an effective treatment for HD has remained elusive, current studies using transplants of bone-marrow-derived mesenchymal stem cells provides considerable promise. (biomedcentral.com)
  • WO 2016/135723 to the present inventors discloses mammalian bone marrow cells enriched with mitochondria for treatment of mitochondrial diseases. (justia.com)
  • The method involves microinjecting heterologous mitochondria into an oocyte or embryonic cell wherein the heterologous mitochondria are capable of achieving at least normal levels of mitochondrial membrane potential in the oocyte or embryonic cell. (justia.com)
  • WO 2017/124037 relates to compositions comprising isolated mitochondria or combined mitochondrial agents and methods of treating disorders using such compositions. (justia.com)
  • In this review, we summarized the mechanism of mitochondrial transfer in the cardiovascular system and outlined the fate and functional role of donor mitochondria. (frontiersin.org)
  • Given the complexity of the biological function of mitochondria, researchers have begun to consider rescuing the injured cells through mitochondrial transfer, that is, replacing damaged mitochondria with healthy mitochondria from donor cells. (frontiersin.org)
  • They demonstrated that transferring functional mitochondria of bone marrow-derived stem cells to defective parenchymal cells increases the aerobic respiration capacity of recipient mitochondria ( 7 ). (frontiersin.org)
  • Because mitochondria can also be transferred via tunneling nanotubes, we hypothesized that HSPC transplantation could also treat mitochondrial diseases. (ucsd.edu)
  • Furthermore, biochemical analysis of mitochondrial respiration, respiratory control rate (RCR) and mitochondrial permeability transition (MPT) supported that HBOT protected mitochondria during liver regeneration. (revitalife.co.in)
  • Multiple research groups have developed artificial mitochondrial transfer/transplantation (AMT/T) methods that transfer healthy mitochondria into damaged cells and recover cellular function. (biomedcentral.com)
  • Collectively, although scientists are increasingly aware of mitochondria's dynamic and transferable capabilities of mitochondria, the unclear mechanism and effectiveness of mitochondrial transfer/transplantation therapy limit its widespread application in diseases. (biomedcentral.com)
  • FRDA is an autosomal recessive mitochondrial disease characterized by neurodegeneration, cardiomyopathy and muscle weakness and patients will be in wheelchair within 10-15 years of onset. (ucsd.edu)
  • Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder caused by mutations in the nuclear TYMP gene, which encodes for thymidine phosphorylase, an enzyme required for the normal metabolism of deoxynucleosides, thymidine, and deoxyuridine. (encyclopedia.pub)
  • We hope this review will provide perspectives on mitochondrial-targeted therapeutics in cardiovascular diseases. (frontiersin.org)
  • Therefore, patients with cardiovascular diseases would fail to achieve the desired outcomes by using these mitochondrial-targeted drugs ( 5 ). (frontiersin.org)
  • She is currently Research Scientist at Stanford University, where she is developing innovative cell therapy approaches to address neuropathic lysosomal storage diseases (LSDs) using hematopoietic stem cell transplantation (HSCT) and genome editing. (stanford.edu)
  • In addition, we are committed to graft engineering and vaccine development to enhance the ability of transplantation to control blood diseases. (harvard.edu)
  • In addition, we also discuss the existing methods of artificial mitochondrial transfer/transplantation (AMT/T) and their therapeutic effects on diseases to explore the potential applications of mitochondrial transfer and transplantation in the treatment of mitochondrial-related diseases, mitochondrial diseases, and cancer. (biomedcentral.com)
  • These data further validate the efficacy of gene therapy for MSUD opening perspectives towards clinical translation. (stanford.edu)
  • Autologous hematopoietic stem cell transplantation using genome-edited cells can become a definitive therapy for hematological and non-hematological disorders with neurological involvement. (stanford.edu)
  • Autologous hematopoietic cell transplantation (HCT) is under investigation as a promising therapy for treatment-refractory MS. Here we identify a reactive myeloid state in chronic experimental autoimmune encephalitis (EAE) mice and MS patients that is surprisingly associated with neuroprotection and immune suppression. (stanford.edu)
  • Data from human trials provides evidence for the efficacy of use of bright light therapy when used as augmentation to standard antidepressants. (greenmedinfo.com)
  • Collectively, our data indicate that MSC therapy rescues impaired mitochondrial morphology, enhances host metabolic capacity, and induces widespread host gene shifting. (frontiersin.org)
  • At present, little is known about the impact of MSC therapy on mitochondrial function and morphology. (frontiersin.org)
  • The specific medications administered depend on the choice of therapy and whether it is supportive care only, immunosuppressive therapy, or hematopoietic cell transplantation. (medscape.com)
  • His work also revealed the complexity of the interaction between iron metabolism, iron therapy, and CKD-mineral and bone disorder in juvenile CKD. (the-asci.org)
  • MSC co-culture with skin fibroblasts from mitochondrial disease patients rescued aberrant mitochondrial morphology from a fission state to a more fused appearance indicating an effect of MSC co-culture on host cell mitochondrial network formation. (frontiersin.org)
  • Because the chromosomal t(14;18) translocation is found in the majority of patients with follicular lymphoma, bone marrow aspiration and chromosome analysis can also help establish the diagnosis. (medscape.com)
  • Most patients with follicular lymphoma present at an advanced stage, and most patients have bone marrow involvement at diagnosis. (medscape.com)
  • Effect of Autologous Conditioned Plasma Injections in Patients With Knee Osteoarthritis. (mayo.edu)
  • The clinical presentation of patients with aplastic anemia includes signs and symptoms related to the decrease in bone marrow production of hematopoietic cells. (medscape.com)
  • In recent years, with a combination of autologous stem-cell transplantation and modern chemotherapy, the 10-y survival of patients presenting at an age less than 60 y is approximately 30% ( 1 , 2 ). (snmjournals.org)
  • Based on the nascent literature on mitochondrial transfer, we address the modes of mitochondrial transfer, their mechanisms, and their roles in physiological and pathological conditions. (biomedcentral.com)
  • Treatment of Mitochondrial Neurogastrointestinal Encephalomyopathy" Encyclopedia , https://encyclopedia.pub/entry/93 (accessed November 30, 2023). (encyclopedia.pub)
  • Time-lapse videography of living cells captures spectacular intracellular mitochondrial movement, and this movement promotes mitochondrial connections to form a dynamic mitochondrial network [ 3 ]. (biomedcentral.com)
  • Intracellular mitochondrial movement is of great importance for cellular functions. (biomedcentral.com)
  • The subsequent elevated systemic concentrations of deoxynucleosides lead to increased intracellular concentrations of their corresponding triphosphates, and ultimately mitochondrial failure due to progressive accumulation of mitochondrial DNA (mtDNA) defects and mtDNA depletion. (encyclopedia.pub)
  • For clinical efficacy, the pressures must be greater than or equal to 1.4 ATA. (revitalife.co.in)
  • RP combined with ophthalmoplegia, dysphagia, ataxia, and cardiac conduction defects is seen in the mitochondrial DNA disorder Kearns-Sayre syndrome (also known as Ragged Red Fiber Myopathy). (wikipedia.org)
  • Korpershoek JV, Rikkers M, Wallis FSA, Dijkstra K, Te Raa M, de Knijff P, Saris DBF , Vonk LA. Mitochondrial Transport from Mesenchymal Stromal Cells to Chondrocytes Increases DNA Content and Proteoglycan Deposition In Vitro in 3D Cultures. (mayo.edu)
  • We also discussed the advantage and challenges of mitochondrial transfer strategies, including cell-based mitochondrial transplantation, extracellular vesicle-based mitochondrial transplantation, and naked mitochondrial transplantation, for the treatment of cardiovascular disorders. (frontiersin.org)
  • However, although clinical trials have demonstrated the safety of MSC treatment, thus far they have not provided evidence for MSC efficacy in the treatment of COPD. (bmj.com)
  • In vivo experiments confirmed mitochondrial abundance and mitochondrial oxygen consumption rates were elevated in host tissues following MSC treatment. (frontiersin.org)
  • This paper reviews intercellular spontaneous mitochondrial transfer modes, mechanisms, and the latest methods of AMT/T. Furthermore, potential application value and mechanism of AMT/T in disease treatment are also discussed. (biomedcentral.com)
  • This tumor-induced diffuse bone loss compounds the treatment-induced bone loss from frequent use of high-dose glucocorticoid medications (typically dexamethasone), leading to severe osteoporosis. (snmjournals.org)
  • Various mitochondrial disorders are known to result from defective genes in the mitochondrial DNA. (justia.com)
  • Concomitantly, a dramatic downregulation of the proximal tubule markers of the megalin-cubilin complex (and some of its target genes involved in mitochondrial biogenesis SCR7 inhibitor database and function (and target genes and and its target genes and was dramatically decreased after LPS injection. (bms-911543.com)
  • These results recommend early and concomitant dysregulation of HNF-1(inhibition of its transcriptional activity without loss of its proteins appearance) and its own focus on genes, aswell as genes involved with mitochondrial function and biogenesis including secreting NKT cells, neutrophils, and monocytes) through regional creation of proinflammatory cytokines, including IFN-and TNF-using IFN-or TNF-stimulation. (bms-911543.com)
  • Studies have shown that cell-to-cell mitochondrial transfer plays an essential role in regulating cardiovascular system development and maintaining normal tissue homeostasis under physiological conditions. (frontiersin.org)
  • can act as donors or recipients during mitochondrial transfer under physiological conditions ( 8 - 12 ). (frontiersin.org)
  • WO 2001/046401 discloses embryonic or stem-like cells produced by cross species nuclear transplantation. (justia.com)
  • Compared with sub-lethal irradiation, Busulfan conditioning enhanced the engraftment of edited CD34+ cells in the bone marrow, as well the long-term homing and survival of bone-marrow-derived cells in viscera, and in the CNS, resulting in higher transgene expression and biochemical correction in these organs. (stanford.edu)
  • We showed that wild-type Hematopoietic Stem and Progenitor Cell (HSPC) transplant in the mouse model of cystinosis, the Ctns -/- mice, led to the abundant tissue integration of bone marrow-derived cells, significant decrease of tissue cystine accumulation, and long-term preservation of the kidney, eye and thyroid function and structure. (ucsd.edu)
  • We also showed for the first time that TNTs could cross the thick, dense and stiff renal tubular basement membrane in vivo and transfer cystinosin-bearing lysosomes to the proximal tubular cells, providing a mechanism underlying the long-term kidney preservation after HSPC transplantation in the Ctns -/- mice. (ucsd.edu)
  • Low-power view of hematoxylin-eosin-stained bone marrow showing hypocellularity, with increased adipose tissue and decreased hematopoietic cells in the marrow space. (medscape.com)
  • Increased Stem cell release from the bone marrow results in increased CD34 cells many folds. (revitalife.co.in)
  • Multiple myeloma (MM) is an incurable malignancy characterized by clonal proliferation of plasma cells in the bone marrow microenvironment. (snmjournals.org)
  • There is no end organ damage in MGUS or in a more progressed condition called smoldering MM. Accordingly, MGUS and smoldering MM are usually not treated, but there are investigational chemoprevention trials attempting to halt the progression to symptomatic MM. In smoldering MM, bone marrow biopsy shows a 10%-60% diffuse infiltration of plasma cells, and the infiltration is less than 10% in MGUS. (snmjournals.org)
  • According to current challenges, there is a need to explore innovative immunotherapies, maximize the tumor-killing efficacy of γδ T cells, and attenuate or eliminate tumor immunosuppression. (explorationpub.com)
  • This study further investigates the efficacy of these transplants with a focus on comparing how passage number of these cells may affect subsequent efficacy following transplantation. (biomedcentral.com)
  • The extent of efficacy of HSPCs to rescue cystinosis was surprising especially considering that cystinosin is a transmembrane lysosomal protein. (ucsd.edu)
  • While cross-correction, either upon secretion- recapture after bone marrow transplantation was shown in several lysosomal storage disorders caused by defective soluble hydrolases, our study is the first demonstration of cross-correction in the context of a lysosomal transmembrane protein and of TNTs as key cellular device in the transfer. (ucsd.edu)
  • FRDA is caused by homozygous GAA repeat expansion mutation within intron 1 of the frataxin gene (FXN) leading to the reduction of expression of the mitochondrial protein frataxin. (ucsd.edu)
  • Much of the morbidity in MM results from insufficiency fractures from the profound bone loss seen in MM. Because the dickkopf 1 protein secreted by MM destroys the osteoblast progenitors ( Fig. 2 ), focal osteolytic bone lesions from MM never heal ( 7 , 9 ). (snmjournals.org)
  • Aplastic anemia is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia (see the image below). (medscape.com)
  • Nuclear transfer efficiency is enhanced by introduction of compatible cytoplasm or mitochondrial DNA (same species or similar to donor cell or nucleus). (justia.com)
  • Application prospect of low-dose porcine anti-thymocyte globulin in HLA-matched sibling donor transplantation. (stembook.org)
  • Hematopoietic stem cell transplantation from an infected SARS-CoV2 haploidentical donor: A case report. (stembook.org)
  • In recent years, a large number of pharmaceutical compounds and nutritional supplements that can boost mitochondrial bioenergetics efficiency have been developed. (frontiersin.org)
  • Here, we show that systemic hematopoietic cell transplantation followed by enhancement of microglia replacement restores microglial function in a Trem2 mutant mouse model of AD. (stanford.edu)
  • The classic imaging finding in MM is one or more focal osteolytic lesions seen on radiographs ( Fig. 1 ) or CT, but there is also a systemic bone loss leading to diffuse osteopenia or osteoporosis ( 7 , 8 ). (snmjournals.org)
  • MM bone loss is due to complex interactions between the tumor and the bone marrow microenvironment leading to both osteoclastic stimulation and inhibition of osteoblastic function. (snmjournals.org)
  • Bone marrow biopsy is performed in addition to aspiration to assess cellularity qualitatively and quantitatively. (medscape.com)
  • Mitochondrial transfer is a novel and intriguing mechanism in intercellular communication. (biomedcentral.com)
  • Collectively, our in vivo results demonstrating 4'-ethynyl-2-fluoro-2'-deoxyadenosine's excellent efficacy in preventing both vaginal and oral HIV transmission together with its relatively low toxicity and high potency against drug-resistant HIV strains support 4'-ethynyl-2-fluoro-2'-deoxyadenosine as a potential PrEP agent for use in women and infants, two of the most vulnerable populations at risk for acquiring HIV. (natap.org)
  • Orthotopic liver transplantation is a beneficial therapeutic option, which shows that restoration of only a fraction of whole-body BCKD enzyme activity is therapeutic. (stanford.edu)
  • In recent years, the phenomenon of mitochondrial transfer has attracted significant attention and interest from biologists and medical investigators. (biomedcentral.com)
  • If a chemical compound targeting one of these hubs is used, it can not only modify the anticipated biological pathways but also change other unexpected mitochondrial processes ( 5 ). (frontiersin.org)
  • Disruption of mitochondrial function by CPI-613 results in a shutdown of ATP and biosynthetic-intermediate production, leading to cancer cell death by apoptosis or necrosis. (bvsalud.org)
  • Multiple myeloma (MM) is an incurable cancer in which uncontrolled plasma cell proliferation disrupts the bone marrow environment and impairs immune function. (hrb.ie)