• Huntington's disease (HD) is a severe autosomal-dominant neurodegenerative disorder caused by a mutation within a gene, encoding huntingtin protein. (frontiersin.org)
  • Huntington's disease (HD) is a severe neurodegenerative pathology characterized by motor dysfunction, cognitive decline and the presence of mental disorders. (frontiersin.org)
  • Here, we studied the impact of IGF2 signaling on protein aggregation in models of Huntington's disease (HD) as proof of concept. (uchile.cl)
  • Researchers generated the first human line of Huntington's disease induced pluripotent stem cells (iPSCs) that do not express the dysfunctional protein underlying the condition. (huntington.cl)
  • Huntington's disease (HD), a severe, inherited neurodegenerative disease that remains without a cure, is caused by an inherited genetic defect in the huntingtin (HTT) gene, which codes for the protein of the same name. (huntington.cl)
  • In this study, researchers generated both mouse and human Huntington's disease iPSCs with a stable silencing of the mutated HTT protein. (huntington.cl)
  • Increasing evidence suggests that early neurodevelopmental defects in Huntington's disease (HD) patients could contribute to the later adult neurodegenerative phenotype. (unipv.it)
  • Huntington's disease (HD) is an incurable inherited brain disorder characterised by massive degeneration of striatal neurons, which correlates with abnormal accumulation of misfolded mutant huntingtin (mHTT) protein. (bvsalud.org)
  • Her post-graduate work focused on the role of neurotrophin receptor signaling and neuroinflammation in Huntington's Disease (HD). (stanford.edu)
  • The model involving injection of quinolinic acid (QA) into the rat striatum simulates many clinical and morphological characteristics of Huntington's disease (HD). (annaly-nevrologii.com)
  • Secondly, we have developed an all-in-one vector-based strategy to convert adult human dermal fibroblasts directly from Huntington's disease (HD) patients and control individuals into induced neurons (iNs). (lu.se)
  • The striatum is the area of the brain that degenerates in Huntington's disease (HD) - a neurodegenerative disorder that as of today, has no cure. (neurodegenerationresearch.eu)
  • Watch the video of Ed Wild's address to the European Huntington's Association annual meeting in September 2012, summarizing how therapies research works and explaining the most exciting ways researchers are trying to study and treat HD. (huntingtonsnswact.org.au)
  • That we hope may slow down the progression of Huntington's disease, or indeed provide new treatments for the symptoms of HD, to give people a better quality of life for longer. (huntingtonsnswact.org.au)
  • To understand how alterations in autophagy contribute to the pathophysiology of chronic neurodegenerative disorders of the CNS, such as Parkinson's disease (PD) or Huntington's disease (HD). (lu.se)
  • Thus, Lgr5+ cells can potentially be a marker for renal stem and/or progenitor cells. (wikipedia.org)
  • Multipotent mouse kidney progenitor cells (MKPC) were obtained from Myh9 targeted mutant mice. (wikipedia.org)
  • Stem/progenitor cells offer the promise that transplantable beta cells may be generated in the laboratory. (ohsu.edu)
  • To deliver on that promise, our laboratory is collaborating with the laboratories of Markus Grompe, M.D., OHSU, and Gordon Keller, Ph.D., University Health Network, Toronto, Canada, in the development and characterization of monoclonal antibodies directed against cell surface molecules expressed by pancreatic cell subsets as well as pancreatic progenitor cells derived from pluripotent stem cells. (ohsu.edu)
  • This project focuses on the compelling need to generate cell-surface marker specific reagents useful for identification, study, and isolation of pulmonary stem/progenitor cells. (ohsu.edu)
  • To investigate human pathogenesis in a physiologically relevant context, we transplanted human pluripotent stem cell-derived neural progenitor cells (hNPCs) from control and HD patients into the striatum of new-born mice. (bvsalud.org)
  • 2017) Differentiation of human telencephalic progenitor cells into MSNs by inducible expression of Gsx2 and Ebf1. (circprot.eu)
  • GRPs are progenitor cells that differentiate into astrocytes and oligodendrocytes following transplantation. (reprocell.com)
  • Firstly, we present a robust 2-week protocol for the differentiation of human pluripotent stem cells (PSCs) into forebrain neural progenitor cells. (lu.se)
  • Recent Accomplishments - Starting in 2013, we incorporated Fluidigm-based multiplex single cell PCR equipment HD Biomark to analyze the differentiation of single cardiac progenitor cells in vitro and found that Nkx2.5 expression marks a subpopulation of committed endocardial precursor cells in the mouse heart (Li et al, Development 2015). (stanford.edu)
  • At first, the mesenchymal stem cell was thought to be the primary component of this undifferentiated cell type, however it is now evident that within the adipose extracellular matrix are also adipocytic precursors (known as progenitor cells) adherent to adipocytes, and in close approximation to a variety of additional undifferentiated multipotent and pluripotent cells, including pericytes and endothelial cells, all thought to play important roles in mesenchymal-stromal derived tissue regeneration. (journalofprolotherapy.com)
  • Here we have used the induced pluripotent stem cell technology to produce patient-specific terminally differentiated GABA-ergic medium spiny neurons modeling a juvenile form of HD (HD76). (frontiersin.org)
  • However, comparing the HD76 neurons with the previously described low-repeat HD models, we have demonstrated that the severity of calcium signaling alterations does not depend on the length of the polyglutamine tract of the mutant huntingtin. (frontiersin.org)
  • Our results are fully supportive in favor of the crucial role of calcium signaling deregulation in the HD pathogenesis and indicate that the cornerstone of excessive calcium uptake in HD-specific neurons is a calcium sensor and store-operated calcium channels activator STIM2, which should become a molecular target for medical treatment and novel neuroprotective drug development. (frontiersin.org)
  • The most vulnerable cells in HD are the striatal medium spiny neurons ( Vonsattel and DiFiglia, 1998 ). (frontiersin.org)
  • Expression of mHTT in astrocytes and neurons is sufficient to increase the brain vessel density in HD mice. (nih.gov)
  • Thus, we aim to test the influence of DNA methylation changes on the in vitro differentiation process of HD-hiPS toward post-mitotic medium sized spiny neurons, in order to check for potential modifications in the methylation patterns. (bmj.com)
  • Methods hiPS from healthy and HD subjects were induced to differentiate toward DARPP32+ neurons through a dual SMAD inhibition step that drives neural induction, BMP/TGFβ inhibition and SHH/DKK1 treatment that generate ventral telencephalic specification. (bmj.com)
  • These results were validated using induced pluripotent stem cells (iPSC)-derived medium spiny neurons from HD patients and spinocerebellar ataxia cases. (uchile.cl)
  • Research on HD has been hampered by the inability to study early dysfunction and progressive degeneration of human striatal neurons in vivo. (bvsalud.org)
  • Remarkably, HD human striatal neurons first developed soluble forms of mHTT, which primarily targeted endoplasmic reticulum, mitochondria and nuclear membrane to cause structural alterations. (bvsalud.org)
  • Furthermore, HD human cells secreted extracellular vesicles containing mHTT monomers and oligomers, which were internalised by non-mutated mouse striatal neurons triggering cell death. (bvsalud.org)
  • The laboratory aims at exploiting the recent advances in embryonic and induced stem cell biology to obtain the medium-sized spiny striatal neurons that degenerate in HD. (circprot.eu)
  • Reconstructing the molecular profile and transcription factors coexpression that qualify human fetal striatal neurons development in vivo (Onorati et al. (circprot.eu)
  • Specifically, UMIL will provide Partner 1 (Lessmann) and Partner 5 (Saudou) with fully characterized differentiated MSNs, carrying different numbers of CAG repeats, aiming at investigate BDNF expression/trafficking parameters by highly innovative microfluidic approaches to isolate axons from cell bodies and follow fluorescently-tagged BDNF trafficking in either pre- or postsynaptic neurons. (circprot.eu)
  • To this purpose, proteome/interactome changes will be evaluated together with Partner 2 (Castren) on drugs-treated cultured HD neurons, in order to evaluate their capability to enhance BDNF expression or mimick specific aspects of TrkB downstream signaling. (circprot.eu)
  • 2013) Developmentally coordinated extrinsic signals drive human pluripotent stem cell differentiation towards fully functional DARPP-32 medium-sized spiny neurons. (circprot.eu)
  • She screened and characterized small molecule ligands for neurotrophin receptors, discovered small molecules that promoted neuronal survival, neurite outgrowth, and differentiation of human stem cells into neurons in vitro. (stanford.edu)
  • These intracellular aggregations of misfolded proteins, such as the nuclear TAR DNA-binding protein of 43 kDa (TDP-43) that accumulates in the cytoplasm, are toxic to the affected neurons and are thought to contribute to neuronal cell death. (atlasantibodies.com)
  • demonstrate through studies on induced pluripotent stem cell-derived cortical neurons that miR-33a is a potential therapeutic target for the treatment of SPG4-related hereditary spastic paraplegia. (portlandpress.com)
  • We identified how striatal neurons mature in the human brain, at a molecular and functional level. (neurodegenerationresearch.eu)
  • Stem cells destined to generate the striatal neurons possess an identifying molecular code, which then turns into a second code acquired by the cells when they move from this location en route to the striatum. (neurodegenerationresearch.eu)
  • To ensure researchers have as much information as possible before moving forward, studies are being done to compare various ways to make striatal neurons, which are the most vulnerable cell type in HD. (huntingtonsnswact.org.au)
  • By means of live cell imaging of fluorescently (GFP) labeled proteins in cultured neurons and isolated brain slices we follow the intracellular transport, synaptic targeting and exocytotic release of these proteins (e.g., neurotrophins) upon physiological stimulation of neuronal cells. (circprot.eu)
  • Depending on the source, stem cells can be classified into two broad categories i.e. embryonic stem cells that are derived from embryos and non-embryonic stem cells that are derived from adult and fetal tissues. (benthamscience.com)
  • Therefore, we aimed to compare the efficiency of using humanized culture conditions, specifically human platelet lysate to fetal bovine serum, for iPS generation from different sources, and to evaluate their stemness. (biomedcentral.com)
  • iPS were generated via a platelet lysate or fetal bovine serum-based culturing protocol from matched dermal, buccal and gingival human fibroblasts, isolated from healthy donors ( n = 2) after informed consent, via episomal plasmid transfection. (biomedcentral.com)
  • Viable offspring derived from fetal and adult mammalian cells. (springer.com)
  • Gene-expression analysis showed that control organoid overlapped with mature human fetal cortical areas, while HD organoids correlated with the immature ventricular zone/subventricular zone. (unipv.it)
  • Extensively revised to incorporate recent research and current clinical practice, The Developing Human: Clinically Oriented Embryology, 11th Edition, covers all aspects of normal and abnormal embryonic and fetal development. (downloadmedicalbook.com)
  • The SC4HD group is standing on the shoulders of giants - learning lots from previous studies that transplanted fetal tissue in the HD brain, changing what didn't work and using what did to move forward logically and safely. (huntingtonsnswact.org.au)
  • Induced pluripotent stem cells (iPS) can be generated from various somatic cells and can subsequently be differentiated to multiple cell types of the body. (biomedcentral.com)
  • To gain pluripotency, the reprogramming process typically involves transfecting adult somatic cells with certain pluripotency markers. (biomedcentral.com)
  • Repair of mitomycin-C-induced chromosomal aberrations was impaired in RAD51B/Rad51b-c.92delT human and mouse somatic cells in vitro and in explanted mouse bone marrow cells. (nature.com)
  • Given that OKSM (Yamanaka) factors convert somatic cells into induced pluripotent stem (iPS) cells, alterations in transcriptional state could affect destiny of the cells. (intechopen.com)
  • Mouse nuclear transfer embryonic stem cells (NT-ESCs) were first established in 2000, and then proved to be able to differentiate either in vivo or in vitro, and give rise to individual tissues through germ line transmission or tetraploid complementation. (benthamscience.com)
  • Embryonic or adult SCs by definition are characterized by their self-renewal and potentiality, peculiarities that allow them to give rise to more SCs and differentiate into various cell lineages under appropriate conditions. (biomedcentral.com)
  • According to their potentiality, embryonic stem cells (ESCs) are classified as totipotent and pluripotent, both of them can differentiate into the three germ lines. (biomedcentral.com)
  • ESCs are obtained from inner cell mass, characterized by unlimited self-renewal and pluripotentiality, which allow them to differentiate into various specialized cells types in a morphologically and functionally way, characteristics that make them attractive for developing differentiated kidney cells (DKCs) [ 9 ]. (biomedcentral.com)
  • The main property that sets ESC apart from other cells is their pluripotent nature, meaning they can give rise/differentiate to cells of the three primary germ layers (mesoderm, endoderm and ectoderm) [ 13 ]. (biomedcentral.com)
  • Furthermore, we have used this protocol to differentiate PSCs from humans and chimpanzees. (lu.se)
  • We show that human and chimpanzee cells differentiate in a similar man¬ner and that the difference in interspecies protein abundance is higher than transcript-level differences, suggesting that post-transcriptional mechanisms play a role in the difference between human and chim¬panzee brain development. (lu.se)
  • The HD iPSC Consortium, (2012) Induced Pluripotent Stem Cells from Patients with Huntington´s Disease: Show CAG Repeat-Expansion-Associated Phenotypes. (circprot.eu)
  • She employs human patient-derived induced pluripotent stem cells ( iPSC s) to elucidate the mechanisms of neuronal cell death in numerous neurodegenerative disorders, including ALS / FTD. (atlasantibodies.com)
  • Experimental neurotransplantation using iPSC derivatives allowance to improve storage of trace memory in rats with QA-induced model of HD, which contributes to correction of cognitive impairments caused by administration of the neurotoxin. (annaly-nevrologii.com)
  • REPROCELL's iPSC-GRPs are the first and only human glial-restricted progenitor (GRP) cell therapeutic. (reprocell.com)
  • It is hoped that the clinical introduction of new therapeutics, such as iPSC-GRPs and Q-Cells® , will improve the quality and length of life for these patients. (reprocell.com)
  • MAGiQ and REPROCELL are working on the research and development of iGRP by combining Q Therapeutics ' technology and know-how on GRP cell with REPROCELL's iPSC technology. (reprocell.com)
  • Chichagova V, Hilgen G , Ghareeb A, Georgiou M, Carter M, Sernagor E , Lako M, Armstrong L. Human iPSC differentiation to retinal organoids in response to IGF1 and BMP4 activation is line- and method-dependent. (neurotree.org)
  • Inhibits proliferation and promotes osteogenic differentiation, while inhibiting adipogenic differentiation, of human amnion-derived mesenchymal stem cells (MSCs) and rabbit bone marrow-derived MSCs (Wu et al. (stemcell.com)
  • Impact of Diabetes Mellitus on Human Mesenchymal Stromal Cell Biology and Functionality: Implications for Autologous Transplantation. (shengsci.com)
  • Multipotent mesenchymal stem/stromal cells (MSCs) have regenerative and immunomodulatory properties to restore and repair injured tissues, making them attractive candidates for cell-based therapies. (shengsci.com)
  • Therefore we have chosen to use the term "adipose-derived stem/stromal cells" (AD-SC's), rather than simply "mesenchymal stem cells. (journalofprolotherapy.com)
  • Moreover, a previously described potential anti-HD drug EVP4593 has been found to attenuate high levels of both huntingtin and STIM2 that may contribute to its neuroprotective effect. (frontiersin.org)
  • At the molecular level, HD occurs due to an increase in the number of CAG repeats in the first exon of the gene encoding the huntingtin protein. (frontiersin.org)
  • Huntington disease (HD) is an inherited neurodegenerative disease caused by the mutant huntingtin gene (mHTT), which harbors expanded CAG repeats. (nih.gov)
  • Cell culture studies revealed that IGF2 treatment decreases the load of intracellular aggregates of mutant huntingtin and a polyglutamine peptide. (uchile.cl)
  • Administration of IGF2 into the brain of HD mice using gene therapy led to a significant decrease in the levels of mutant huntingtin in three different animal models. (uchile.cl)
  • The cells represent a potential resource to investigate the function of the huntingtin protein in vitro (in a lab culture dish or test tube). (huntington.cl)
  • Overall, this gene silencing strategy allowed researchers to establish "the first human induced pluripotent stem cells with stable huntingtin knock-down, and they can be helpful for in vitro research on huntingtin functions in human development," the study concluded. (huntington.cl)
  • These results show that mutant huntingtin precludes normal neuronal fate acquisition and highlights a possible connection between mutant huntingtin and abnormal neural development in HD. (unipv.it)
  • 2012) An evolutionary recent neuroepithelial cell adhesion function of huntingtin implicates ADAM10-Ncadherin. (circprot.eu)
  • We've talked a lot about huntingtin protein clumping up in cells, but Anastasia is looking at the huntingtin message - the recipe - forming clumps in the cell's nucleus. (huntingtonsnswact.org.au)
  • They have added this to their toolkit of compounds which change the levels of important targets in HD including total huntingtin, toxic specific and MSH3, a genetic modifier of HD. (huntingtonsnswact.org.au)
  • These cells are able to incorporate into other renal tissues, and was able to repeatedly form spheres in 3D cultures, and clonal analysis also exhibited its multipotency. (wikipedia.org)
  • Stem cells represent a small fraction of cells located in different tissues. (shengsci.com)
  • this kind of study is important because we need to understand more about how our tissues and our cells develop in order to understand why they degenerate. (neurodegenerationresearch.eu)
  • We also use human stem cells as a test-tube model to study the process of heart formation and to create new human heart tissues and organs from these stem cells. (stanford.edu)
  • Recognition of the vast number of undifferentiated cells associated with the stromal vascular fraction has resulted in extensive research demonstrating the heterogeneity of such cells, and their ability to participate in production of all mesodermal-derived tissues. (journalofprolotherapy.com)
  • The histiocytoses encompass a group of diverse disorders characterized by the accumulation and infiltration of variable numbers of monocytes, macrophages, and dendritic cells in the affected tissues. (medscape.com)
  • Dendritic cells display a large amount of MHC-peptide complexes at their surface and can increase the expression of costimulatory receptors and migrate to the lymph nodes, spleen, and other lymphoid tissues, where they activate specific T cells. (medscape.com)
  • Therefore, we sought to systemically track human-derived ENCC in a mouse model with an intact enteric nervous system. (eventscribe.net)
  • Incorporating microglia-like cells in human induced pluripotent stem cell-derived retinal organoids. (neurotree.org)
  • Renal stem cells are self-renewing, multipotent stem cells which are able to give rise to all the cell types of the kidney. (wikipedia.org)
  • With high levels of platelet-derived growth factors and cytokines, this combination provides both a living bioscaffold and a multipotent cell replenishment source useful for enhanced musculoskeletal healing. (journalofprolotherapy.com)
  • Astrocytes from HD mice and patients contained more VEGF-A, which triggers proliferation of endothelial cells and may be responsible for the augmented neurovascular changes. (nih.gov)
  • Decreased proliferation and Nanog expression in mouse embryonic stem (ES) cells, with increased erythroid differentiation (Chae et al. (stemcell.com)
  • Gene expression must be appropriately maintained to regulate development, differentiation, and proliferation of cells. (intechopen.com)
  • This suggests the possibility that strictly regulated self-renewal mediated by Wnt signaling in cancer cells may be disturbed by the SUMOylation pathway to allow more malignant proliferation. (frontiersin.org)
  • Each category of histiocytosis can be traced to reactive or neoplastic proliferation in one of these cell lineages. (medscape.com)
  • Here, by using HD-derived induced pluripotent stem cell lines, we report that early telencephalic induction and late neural identity are affected in cortical and striatal populations. (unipv.it)
  • Furthermore, UMIL will also focus on mechanisms of optimization of BDNF/TrkB signaling networks as endogenous protective factors against HD, aiming at restore the cortical axon and striatal dendritic defects in HD. (circprot.eu)
  • These DMRs could be considered as unique and specifically associated to HD phenotype. (bmj.com)
  • Further studies will allow to elucidate the real contribution of DNA methylation in HD pathogenesis and phenotype. (bmj.com)
  • In the present work, we have analyzed the influence of two Ti implant surfaces characterized by similar microtopography but different nanotopography and chemistry on the osteoblastic phenotype of Dental Pulp Stem Cells (DPSCs). (shengsci.com)
  • We were interested in the developmental potential of single cardiac progenitor cell into cardiomyocytes, smooth muscle cells, and endothelial cells. (stanford.edu)
  • DNA methylation influences gene expression, embryonic development and cell fate commitment. (bmj.com)
  • Several genome-wide studies have reported genomic variants that correlate with gene expression levels in various tissue and cell types. (biomedcentral.com)
  • This focuses on the expression of the human tdp1 mutant gene in brain tissue of flies lacking the endogenous Gkt protein. (ataxia.org)
  • On the other hand, the lab is heavily involved in the study of the molecular mechanisms underlying neuronal cell loss as a consequence of the mutation in the HD gene and has identified a number of molecular pathways targeted by the mutant gene. (circprot.eu)
  • In October 2020, Amira was appointed Instructor of Neurodegenerative Disease Research at Stanford Neurology, to help develop improved and more powerful approaches that will better reveal key synaptic mechanisms and candidate modules associated with neuroplasticity and affected in AD mouse models, by identifying activity-dependent gene expression signatures that will ultimately serve as a platform for potential therapeutics and animal to human translation. (stanford.edu)
  • Recent global analyses of gene transcripts revealed that specific transcription factors (TFs) and their networking systems physiologically correspond to the onset of human diseases, including cancer. (intechopen.com)
  • We show that a large CAG expansion causes complete failure of the neuro-ectodermal acquisition, while cells carrying shorter CAGs repeats show gross abnormalities in neural rosette formation as well as disrupted cytoarchitecture in cortical organoids. (unipv.it)
  • Since shRNA-mediated suppression of STIM2 decreased store-operated calcium uptake, we have speculated that high expression of STIM2 underlies the excessive entry through store-operated calcium channels in HD pathology. (frontiersin.org)
  • We studied human embryonic stem cells (hESCs) and human induced pluripotent stem cells (hiPSCs) measuring the SNPs and CNVs with Affymetrix SNP 6 microarrays and expression values with Affymetrix Exon microarrays. (biomedcentral.com)
  • Our results revealed that in the human pluripotent stem cells, the expression values of several genes, transcripts and exons were affected due to the genomic variation. (biomedcentral.com)
  • Embryonic stem cells (ESC) can be expanded indefinitely without undergoing replicative senescence or aging due to their high telomerase expression. (biomedcentral.com)
  • Activation of muscle-specific genes in pigment, nerve, fat, liver, and fibroblast cell lines by forced expression of MyoD. (springer.com)
  • Decellularised extracellular matrix-derived peptides from neural retina and retinal pigment epithelium enhance the expression of synaptic markers and light responsiveness of human pluripotent stem cell derived retinal organoids. (neurotree.org)
  • We subsequently became interested in developing a developing heart expression atlas in order to identify the cell type, developmental stage, and anatomical location of each single cardiac cells during mouse heart development using a random forest-based computational algorithm that we termed ATLAS-seq. (stanford.edu)
  • Each reagent was used to transfect HEK 293, HeLa, LNCaP, HepG2, and A549 cell lines in a 96-well format, and GFP expression was analyzed 48 hours post-transfection. (thermofisher.com)
  • In fact, "studies in induced pluripotent stem cells in Huntington disease models have demonstrated that multiple molecular processes are altered by the mutant HTT protein and suggested its silencing as a promising therapeutic strategy," researchers wrote. (huntington.cl)
  • Somos un grupo de personas, que en nuestras familias o gente cercana, se ha presentado la enfermedad de Huntington. (huntington.cl)
  • The lab's ultimate goal is to identify cells, molecules and pathways that are suitable for therapeutic intervention and new reagents for drug screening in Huntington´s Disease. (circprot.eu)
  • Demyelinating conditions include ALS, TM, cerebral palsy (CP), cerebral infarction (Stroke) and multiple sclerosis (MS). Neurodegenerative conditions include Huntington disease (HD), spinal cord injury (SCI), traumatic brain injury (TBI), Parkinson disease (PD) and Alzheimer disease (AD). (reprocell.com)
  • Embryonic stem cells (ESCs) can grow infinitely and give rise to all types of cells in human body, thus of tremendous therapeutic potentials for a variety of diseases, such as Parkinson's disease, spinal cord injury, and diabetes. (benthamscience.com)
  • What is more, by deriving NT-ESCs from patient cells, the problem of immune rejection may be avoided. (benthamscience.com)
  • However, the derivation of human NT-ESCs goes with the destruction of clone embryos, leading to fierce ethical disputes. (benthamscience.com)
  • The resultant impaired VR might hinder cerebral hemodynamics and increase brain atrophy during HD progression. (nih.gov)
  • We conclude that interaction of mHTT soluble forms with key cellular organelles initially drives disease progression in HD patients and their transmission through exosomes contributes to spread the disease in a non-cell autonomous manner. (bvsalud.org)
  • Accordingly, Rad51b-c.92delT variant reduced replication fork progression of patient-derived lymphoblastoid cell lines and pluripotent reprogramming efficiency of primary mouse embryonic fibroblasts. (nature.com)
  • Anastasia thinks this toolkit is an excellent portfolio of different options for targeting HD, which may also help us unpick exactly which protein or protein form is important in the disease progression. (huntingtonsnswact.org.au)
  • Furthermore, the Wnt/β-catenin can crosstalk with other signaling pathways including Notch, FGF, Hedgehog, and TGF-β/BMP signaling cascades to form a signaling network to regulate the survival and progression of cancer cells ( 14 - 16 ). (frontiersin.org)
  • Recent studies suggest that brain cell type specific intracellular environments may play important roles in the generation of structurally different protein aggregates that define neurodegenerative diseases. (biomedcentral.com)
  • Using human induced pluripotent stem cells (hiPSC) and biochemical and vibrational spectroscopy techniques, we studied whether Parkinson's disease (PD) patient genomes could modulate alpha-synuclein (aSYN) protein aggregates formation. (biomedcentral.com)
  • Importantly, we discovered that aSYN protein aggregation is modulated by patient brain cells' intracellular milieus at the primary nucleation phase. (biomedcentral.com)
  • However, there is no data that indicates if and how patient-specific brain cells' intracellular milieus modulate protein aggregation. (biomedcentral.com)
  • Here, we studied changes in intracellular environment of PD patients' brain cells and how they modulate aSYN protein aggregation, using human induced pluripotent stem cells (hiPSC) and biochemical methods combined with infrared spectroscopy technique. (biomedcentral.com)
  • This study identifies IGF2 as a relevant factor deregulated in HD, operating as a disease modifier that buffers the accumulation of abnormal protein species. (uchile.cl)
  • They also need a protein's assistance, like a channel protein or some other transmembrane protein, to cross the cell membrane. (atlasantibodies.com)
  • In line with our previous results, we found that there are marked differences between HD patients and controls at the protein level but not at the transcriptional level. (lu.se)
  • We have more recently acquired the expertise to execute multi-omic technologies based on single cell microfluidic tools developed from 10x Genomics including single nuclear ATAC-seq with RNA-seq, single cell TCR sequencing with feature barcoding of surface protein (i.e. (stanford.edu)
  • Each reagent was used to transfect HeLa cells in a 96-well format at the indicated doses with an emerald green fluorescent protein (GFP)-expressing vector. (thermofisher.com)
  • Signaling is initiated when the Wnt ligand binds to the Frizzled receptor on the cell membrane and the LDL receptor-associated protein 5/6 (LRP5/6) co-receptor. (frontiersin.org)
  • Induces upregulation of the pluripotency network genes (Klf4, Klf2, Nanog, Oct4, Myc, Sox2) and epigenetic-associated proteins (Dnmt3a, Mbd3) in mouse ES cells, and inhibits retinoic acid-induced differentiation (Adamo et al. (stemcell.com)
  • NANOGP1, a tandem duplicate of NANOG, exhibits partial functional conservation in human na ve pluripotent stem cells. (qcfail.com)
  • whole well image of culture well (96 well plate format) containing human induced pluripotent stem cells grown on mouse feeders, stained for pluripotent markers NANOG (red) and OCT4 (green). (lu.se)
  • Since rodent models cannot recapitulate many of the human disease features, human induced pluripotent stem cells derived from Parkinson's patients have been used to generate brain organoids, greatly contributing to our understanding of the disease pathophysiology. (nature.com)
  • To understand the multifaced aspects of Parkinson's disease, it may be desirable to expand the complexity of these models, to include different brain regions, vasculature, immune cells as well as additional diverse organ-specific organoids such as gut and intestine. (nature.com)
  • Human Retinal Organoids Provide a Suitable Tool for Toxicological Investigations: A Comprehensive Validation Using Drugs and Compounds Affecting the Retina. (neurotree.org)
  • Georgiou M, Chichagova V, Hilgen G , Dorgau B, Sernagor E , Armstrong L, Lako M. Room temperature shipment does not affect the biological activity of pluripotent stem cell-derived retinal organoids. (neurotree.org)
  • Sozzi E, Nilsson F, Kajtez J, Parmar M, Fiorenzano A. Generation of Human Ventral Midbrain Organoids Derived from Pluripotent Stem Cells . (lu.se)
  • We previously reported that the brain vessel density is higher in mice and patients with HD than in controls. (nih.gov)
  • However, the supply of donor-derived islets will never meet the needs of the large number of patients who could potentially benefit from this cell-based therapy. (ohsu.edu)
  • Moreover, analysis of human postmortem brain tissue and blood samples from HD patients showed a reduction in IGF2 level. (uchile.cl)
  • Comparative characterization of human induced pluripotent stem cells (hiPSC) derived from patients with schizophrenia and autism. (sylics.com)
  • Parkinson's disease patients can find hope in a new treatment, thanks to stem cell research that successfully replaces damaged nerves. (neurodegenerationresearch.eu)
  • Recently, we have begun to address the pathogenic immune response in patients with myocarditis from treatment with immune checkpoint inhibitors (ICIs) for cancer therapy using single cell immunology tools from 10x Genomics. (stanford.edu)
  • 2014) Molecular and functional definition of the developing human striatum. (circprot.eu)
  • The effects of neurotransplantation on reproduction of the conditioned passive avoidance reflex were studied in rats with the model of HD induced by injection of QA into the caudate nuclei of the striatum. (annaly-nevrologii.com)
  • It took 4 years of continuous experiments of 17 researchers from 6 groups in 2 European countries to understand more about how cells develop in the striatum. (neurodegenerationresearch.eu)
  • Then, a third identifying code is acquired when the cells finally reach the striatum, where they will remain. (neurodegenerationresearch.eu)
  • Our results revealed altogether 217 genes and 584 SNPs whose genomic alterations affect the transcriptome in the same cells. (biomedcentral.com)
  • After sequencing the human genome, numerous projects have focused on characterizing genomic alterations and associating them with the different diseases or functional elements of the genome. (biomedcentral.com)
  • Areas of research focus are beta cell replacement therapies for the treatment of diabetes, the identification of lung progenitor cell populations involved in lung repair, and in the diagnosis of pancreatic cancer. (ohsu.edu)
  • and 3) facilitate the purification of cell culture expanded cell populations for cell-based therapies. (ohsu.edu)
  • Biodistribution studies are critical for the application of cellular techniques for future human therapies. (eventscribe.net)
  • Now I come to review this slide, I realise actually that I'm doing therapies as number three and stem cells as number four, so that was really just to make sure you were concentrating. (huntingtonsnswact.org.au)
  • In this chapter, revisiting known TFs, we would argue if transcription controlling strategies could contribute for the novel therapies on human diseases. (intechopen.com)
  • There's still a lot to work out before we have this technology in humans for HD, but stem cells represent a very powerful source for cell replacement therapies. (huntingtonsnswact.org.au)
  • Wnt/β-catenin signaling is a delicate and complex signal transduction pathway mediated by multiple signaling molecules, which plays a significant role in regulating human physiology and pathology. (frontiersin.org)
  • Such a description excludes diseases in which infiltration of these cells occurs in response to a primary pathology. (medscape.com)
  • Improved understanding of the pathology of histiocytic disorders requires knowledge of the origins, biology, and physiology of the cells involved. (medscape.com)
  • She employs human patient-derived induced pluripotent stem cells to elucidate the nuclear-cytoplasmic trafficking mechanisms in neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). (atlasantibodies.com)
  • Moreover, an astrocytic inflammatory response, which reduces the survival of pericytes through an IκB kinase-dependent pathway, mediates the low pericyte coverage of blood vessels in HD brains. (nih.gov)
  • Our findings suggest that the inflammation-prone HD astrocytes provide less pericyte coverage by promoting angiogenesis and reducing the number of pericytes and that these changes can explain the inferior VR in HD mice. (nih.gov)
  • Searching for effective treatment methods is rather topical because of the fatality of HD. (annaly-nevrologii.com)
  • To circumvent these obstacles, we have developed two methods for the investigation of human neural cells in culture. (lu.se)
  • HTT silencing resulted in moderate effects on Wnt and MAPK pathways in both mouse and human HD induced pluripotent stem cells. (huntington.cl)
  • His research team is developing new drugs that are focused on the modulation of fundamental cell signaling pathways that are involved in neurodegeneration. (stanford.edu)
  • Dendritic cells appear to develop in several pathways. (medscape.com)
  • Kuldip S. Sidhu , " Frontiers in Pluripotent Stem Cells Research and Therapeutic Potentials Bench-to-Bedside ", Bentham Science Publishers (2012). (benthamscience.com)
  • In 2003 he became Director of the OHSU Hematopoietic Cell Processing Laboratory, and in 2004 he became a member of the Oregon Stem Cell Center, where he is Director of the Center's monoclonal antibody and flow cytometry core facilities and conducts independent research. (ohsu.edu)
  • In this research, we generate differentiated kidney cells (DKCs) from mouse pluripotent stem cells (mPSCs) analyzing their morphological, genetic, phenotypic, and spectroscopic characteristics along differentiation, highlighting that there are no reports of the use of Fourier transform infrared (FTIR) spectroscopy to characterize the directed differentiation of mPSCs to DKCs. (biomedcentral.com)
  • Evans MD, Kelley J. US attitudes toward human embryonic stem cell research. (springer.com)
  • The overall theme of her current research concentrates on studying the roles and mechanisms of small molecules in neuronal networks, synaptic functions, neural stem cells and neurodegenerative diseases which include Alzheimer's disease (AD), Parkinson's disease (PD) and Charcot-Marie-Tooth (CMT) disease. (stanford.edu)
  • She joined Dr. Frank Longo's laboratory as a Senior Scientist in the Stanford School of Medicine in 2008, where her research has focused on identifying mechanisms underlying HD and Alzheimer's disease (AD) and developing small molecule therapeutic strategies to target these mechanisms. (stanford.edu)
  • Innovations in Training: Toward Mitigating "Eternal post-docdum" in Stem Cell Research. (shengsci.com)
  • A holistic strategy to prevent malnutrition should be developed, and could include research on the role of, amongst other, nutrients in the human organism (their bioavailability and interactions), the gut microbiome, food preparation at home, the physical and social environment (such as social networks). (iuphar.org)
  • Modern research in animal model organisms and humans indicates that autophagy declines during ageing. (mdpi.com)
  • More so, TEs are a rich source of genetic variation, which makes them an intriguing research avenue to investigate humanspecific traits, including their impact on human brain evolution and their relevance in disease. (lu.se)
  • In a guest post, Prof. Elena Cattaneo, University of Milan, Italy reports in EUROSTEMCELL on recent research that examines how a particular type of cell develops in the human brain, and how studies like this fit into the overall picture of research collaboration and funding, in Italy and in Europe. (neurodegenerationresearch.eu)
  • Stem cells can be used in HD research for various purposes: either as a tool to understand more about HD or, perhaps, as a therapy. (huntingtonsnswact.org.au)
  • Martin GR. Isolation of a pluripotent cell line from early mouse embryos cultured in medium conditioned by teratocarcinoma stem cells. (springer.com)
  • Establishment in culture of pluripotential cells from mouse embryos. (springer.com)
  • Induced pluripotent stem cells differentiated into ENCC were labeled with a fluorescent dye, Di-I. 100υl of media alone or with 1e7 cells were injected into the stomach subserosa of a NOD-SCID mouse (n= 2 control, 6 experimental). (eventscribe.net)
  • Assessment of Behavioral Characteristics With Procedures of Minimal Human Interference in the mdx Mouse Model for Duchenne Muscular Dystrophy. (sylics.com)
  • Hilgen G , Lockwood E, Ratcliff JEJ, Sernagor E . Pan-retinal characterization of parvalbumin-calretinin co-expressing cells in the mouse retina F1000research . (neurotree.org)
  • To understand the functional analyses of miRNAs and their role in neural stem cells by characterizing and identifiying known and novel RNAs bound to the mouse and human RNA induced Silencing Complex in various cell types of the brain. (lu.se)
  • Transplantation of living nuclei from blastula cells into enucleated frogs' eggs. (springer.com)
  • In particular, this can be performed by transplantation of cells capable of undergoing neuronal differentiation and integration into the proper structural and functional brain networks. (annaly-nevrologii.com)
  • These include various infections, hematologic malignancies, inorganic dust exposure (silica, aluminum, or titanium), and immunodeficiency states such as infection with human immunodeficiency virus ( HIV ) and lung transplantation . (medscape.com)
  • In mammals, functional analysis of the individual RAD51 paralogues in cell lines has shown similar but non-redundant contributions in DNA repair processes such as HR efficiency, RAD51 nuclear focus formation, sensitization to mitomycin C (MMC) and protection of perturbed replications forks [ 11 ]. (nature.com)
  • Hilgen G , Kartsaki E, Kartysh V, Cessac B, Sernagor E . A novel approach to the functional classification of retinal ganglion cells. (neurotree.org)
  • Sarcomere Shortening of Pluripotent Stem Cell-Derived Cardiomyocytes using Fluorescent-Tagged Sarcomere Proteins. (jove.com)
  • Using in vivo lineage tracing techniques, Lgr5+ cells were found to contribute to the nephron, specifically to the ascending limb of the loop of Henle and the distal convoluted tubule. (wikipedia.org)
  • She further discovered significant therapeutic properties of those small molecules that prevented neuronal degeneration, dendrites dystrophy and spine loss in both diseases of in vitro cell models and in vivo animal models. (stanford.edu)
  • Regarding vibrational spectroscopy analysis by FTIR, bands related with biomolecules were shown on mPSCs and DKCs spectra, observing distinct differences between cell lineages and maturation stages. (biomedcentral.com)
  • Results Global CpG methylation showed statistical significant differences in the methylation percentages of HD-iPS compared to control-iPS during the differentiation process. (bmj.com)
  • The comparative study of metabolomes of these cells is important for understanding the differences in metabolism between somatic and pluripotent cells, and also the possibl. (shengsci.com)
  • Induced pluripotent stem cells, another types of pluripotent stem cells derived from any tissue by reprogramming and are the homologous source of stem cells. (benthamscience.com)
  • Our laboratory, in collaboration with the laboratory of Jeffrey Whitsett, M.D., at the Cincinnati Children's Hospital, is generating and characterizing a panel of monoclonal antibodies targeting cell-surface markers on different subsets of pulmonary cells involved in the process of pulmonary tissue regeneration. (ohsu.edu)
  • Neuroscience focusing on human development and disease has long been hampered due to ethical rea¬sons, low tissue availability, and low translatability from animal models. (lu.se)
  • One explanation for deficient repair is when undifferentiated adult stem repair cells are inadequate in number or cannot be stimulated within the damaged tissue site. (journalofprolotherapy.com)
  • With improved understanding of tissue healing and regeneration, stem cell Prolotherapy is gaining significant clinical importance and potential. (journalofprolotherapy.com)
  • In the 1990's platelet rich plasma (PRP) gained acceptance in many surgical circles, and in the 2000's, Prolotherapists and other physicians in the orthopedic and sports medicine field, began using high-density PRP concentrates (HD-PRP), defined as a minimum of 4 times patient baseline platelet levels, to stimulate musculoskeletal connective tissue repair. (journalofprolotherapy.com)
  • A Standard curves from 11 miRNAs expressed in human muscle tissue. (svbww.eu)
  • The developmental capacity of nuclei taken from differentiating endoderm cells of Xenopus laevis. (springer.com)
  • The developmental capacity of nuclei taken from intestinal epithelium cells of feeding tadpoles. (springer.com)
  • The developmental capacity of nuclei transplanted from keratinized skin cells of adult frogs. (springer.com)
  • The cell nuclei is stained with DAPI (blue). (lu.se)
  • The motor symptoms are attributed to dopaminergic cell loss within the substantia nigra (SN) pars compacta, resulting in subsequent dysfunction of the basal ganglia, a cluster of deep nuclei that participate in the initiation and execution of movements [ 3 ]. (nature.com)
  • Injection of MKPC into mice post-ischemic injury saw the MKPC regenerating different cell lineages and was able to regenerate renal function and enhanced survival. (wikipedia.org)
  • Human induced pluripotent stem cells (hiPS) constitute an appropriate cellular model that can be exploited to mimic and recapitulate brain development. (bmj.com)
  • This is because Drosophila genes controlling fundamental cellular functions, such as cell growth and death, are quite identical to those found in human cells. (ataxia.org)
  • BDNF-GFP), over cellular neurobiology (electrophysiology and live cell imaging) up to bahavioral analysis. (circprot.eu)
  • Studies have shown that the Wnt/β-catenin signaling pathway controls cell fate and function through the transcriptional and post-translational regulation of omics networks. (frontiersin.org)
  • Lipofectamine 3000 reagent outperforms Invitrogen Lipofectamine 2000 and FuGENE HD reagents. (thermofisher.com)
  • Lipofectamine 3000 reagent provided higher GFP transfection efficiency than Lipofectamine 2000 and FuGENE HD reagents for all five cell lines. (thermofisher.com)
  • Zerti D, Hilgen G , Dorgau B, Collin J, Ader M, Armstrong L, Sernagor E , Lako M. Transplanted pluripotent stem cell-derived photoreceptor precursors elicit conventional and unusual light responses in mice with advanced retinal degeneration. (neurotree.org)