Abstract:
BACKGROUND:Amyotrophic lateral sclerosis (ALS) represents a devastating, progressive, heterogeneous, and the most common motor neuron (MN) disease. To date, no cure has been available for the condition. Studies with transgenic mice have yielded significant results that help us understand the underlying mechanisms of ALS. Nonetheless, none of more than 30 large clinical trials over the past 20 years proved successful, which led some researchers to challenge the validity of the preclinical models. METHODS:Human-induced pluripotent cells (iPSCs) were established by introducing Sendai virus into fibroblast cells. We established TDP-43 HES by inserting CAG-TDP43 (G298S) cassette or the CAG-EGFP cassette into PPP1R12C-locus of human embryonic stem cells (ESC, H9) by TALEN-mediated homologous recombination. iPSCs or HESC were differentiated to motor neurons and non-motor neuron as control. Relevant biomarkers were detected in different differentiated stages. TDP-43 aggregates, neurofilament, and mitochondria analyses were performed. RESULTS:In this study, using iPSCs-derived human MN from an ALS patient with a TDP43 G298S mutation and two sporadic ALS patients, we showed that both sporadic and familial ALS were characterized by TDP-43 aggregates in the surviving MN. Significantly higher neurofilament (NF) inclusion was also found in ALS MN compared with wild-type (WT) GM15 controls (P < 0.05). The neurite mitochondria density was significantly lower in ALS MN than that in the control MNs. Transgenesis of TDP-43 G298S into AAVS locus in human embryonic stem cells reproduced phenotype of patient-derived G289S MN. By challenging MNs with a proteasome inhibitor, we found that MNs were more vulnerable to MG132, with some accompanying phenotype changes, such as TDP43 translocation, NF inclusion, mitochondria distribution impairment, and activation of caspase3. CONCLUSIONS:Our results suggested that changes in TDP43 protein, NF inclusion, and distribution impairment of mitochondria are common early pathology both in familial and sporadic ALS. These findings will help us gain insight into the pathogenesis of the condition and screen relevant drugs for the disease.
journal_name
Stem Cell Res Therjournal_title
Stem cell research & therapyauthors
Sun X,Song J,Huang H,Chen H,Qian Kdoi
10.1186/s13287-018-1048-1subject
Has Abstractpub_date
2018-11-15 00:00:00pages
315issue
1issn
1757-6512pii
10.1186/s13287-018-1048-1journal_volume
9pub_type
杂志文章abstract:BACKGROUND:Neonatal hypoxia-ischemia induces massive brain damage during the perinatal period, resulting in long-term consequences to central nervous system structural and functional maturation. Although neural progenitor cells (NPCs) migrate through the parenchyma and home in to injury sites in the rodent brain, the m...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-017-0474-9
更新日期:2017-02-07 00:00:00
abstract:BACKGROUND:Bone marrow mesenchymal stem cells (BMMSCs) are suitable cell sources for dental pulp regeneration, but the mechanism of BMMSCs differentiation into odontogenic lineage remains unknown. The aim of the present study was to reveal the role of magnesium transporter protein 1 (MagT1) and MAPK pathways in the odo...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-019-1148-6
更新日期:2019-01-31 00:00:00
abstract:INTRODUCTION:Delivered systemically or natively circulating mesenchymal stem cells accumulate in injured tissues. During homing mesenchymal stem cells adhere to endothelial cells and infiltrate underlying tissue. Previously we have shown that adhesiveness of endothelial cells for mesenchymal stem cells correlates with ...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/scrt35
更新日期:2010-11-17 00:00:00
abstract:BACKGROUND:Adult full-thickness cutaneous wound repair suffers from an imbalanced immune response, leading to nonfunctional reconstructed tissue and fibrosis. Although various treatments have been reported, the immune-mediated tissue regeneration driven by biomaterial offers an attractive regenerative strategy for dama...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-018-0768-6
更新日期:2018-01-31 00:00:00
abstract:INTRODUCTION:Mesenchymal stem cells (MSCs) are multipotent and have been derived from various tissues. Although MSCs share many basic features, they often display subtle tissue specific differences. We previously demonstrated that bone marrow (BM) MSCs frequently become polyploid in culture. This tendency was mediated ...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/scrt529
更新日期:2014-12-17 00:00:00
abstract:INTRODUCTION:Bone marrow (BM) stroma currently represents the most common and investigated source of mesenchymal progenitor cells (MPCs); however, comparable adult progenitor or stem cells have also been isolated from a wide variety of tissues. This study aims to assess the functional similarities of MPCs from differen...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/scrt11
更新日期:2010-05-04 00:00:00
abstract:BACKGROUND:Precision or individualized medicine is increasingly pervasive across medical and surgical disciplines. However, the concept has not been introduced in regenerative medicine. MAIN BODY:Targeted and engineered cellular and acellular therapy, specific to individuals or disease states, could significantly impr...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-02092-w
更新日期:2021-01-07 00:00:00
abstract:BACKGROUND:Complex immunophenotypic repertoires defining discrete adipose-derived stem cell (ASC) subpopulations may hold a key toward identifying predictors of clinical utility. To this end, we sorted out of the freshly established ASCs four subpopulations (SPs) according to a specific pattern of co-expression of six ...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-016-0435-8
更新日期:2016-12-01 00:00:00
abstract:BACKGROUND:Mesenchymal stromal cells (MSCs) are an attractive therapeutic agent in regenerative medicine. Recently, there has been a paradigm shift from differentiation of MSCs to their paracrine effects at the injury site. Several reports elucidate the role of trophic factors secreted by MSCs toward the repair of inju...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-017-0752-6
更新日期:2018-02-06 00:00:00
abstract:BACKGROUND:Hertwig's epithelial root sheath (HERS) is important in guiding tooth root formation by differentiating into cementoblasts through epithelial-mesenchymal transition (EMT) and inducing odontoblastic differentiation of dental papilla through epithelial-mesenchymal interaction (EMI) during the tooth root develo...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-018-1106-8
更新日期:2019-01-03 00:00:00
abstract:BACKGROUND:Mesenchymal stem cells, including those derived from human adipose tissue (hASCs), are currently being widely investigated for cell therapy. However, when transplanted at the site of injury, the survival and engraftment rates of hASCs are low, mainly due to the harsh microenvironment they encounter, characte...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-01851-z
更新日期:2020-08-03 00:00:00
abstract:BACKGROUND:Neural stem cells for the treatment of spinal cord injury (SCI) are of particular interest for future therapeutic use. However, until now, stem cell therapies are often limited due to the inhibitory environment following the injury. Therefore, in this study, we aimed at testing a combinatorial approach with ...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-015-0268-x
更新日期:2016-01-13 00:00:00
abstract::The discovery of induced pluripotent stem cells (iPSCs) in 2006 was a major breakthrough for regenerative medicine. The establishment of patient-specific iPSCs has created the opportunity to model diseases in culture systems, with the potential to rapidly advance the drug discovery field. Current methods of drug disco...
journal_title:Stem cell research & therapy
pub_type: 杂志文章,评审
doi:10.1186/scrt363
更新日期:2013-01-01 00:00:00
abstract::Over the past decade a lot of research has been performed towards the therapeutic use of mesenchymal stem cells (MSCs) in neurodegenerative and neuroinflammatory diseases. MSCs have shown to be beneficial in different preclinical studies of central nervous system (CNS) disorders due to their immunomodulatory propertie...
journal_title:Stem cell research & therapy
pub_type: 杂志文章,评审
doi:10.1186/scrt312
更新日期:2013-08-22 00:00:00
abstract::The original article [1] contained errors in the presentation of Figure 7. ...
journal_title:Stem cell research & therapy
pub_type: 已发布勘误
doi:10.1186/s13287-018-1116-6
更新日期:2019-01-08 00:00:00
abstract:BACKGROUND:Human adipose-derived stromal cells (hASCs) have been gaining increasing popularity in regenerative medicine thanks to their multipotency, ease of collection, and efficient culture. Similarly to other stromal cells, their function is particularly sensitive to the culture conditions, including the composition...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-019-1333-7
更新日期:2019-08-14 00:00:00
abstract:INTRODUCTION:Adipose-derived stroma cells (ASCs) are attractive cells for cell-based gene therapy but are generally difficult to transfect. Nucleofection has proven to be an efficient method for transfection of primary cells. Therefore, we used this technique to transfect ASCs with a vector encoding for Ambystoma mexic...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/scrt503
更新日期:2014-10-09 00:00:00
abstract::The efficacy of tendon-derived stem cells (TDSCs) for the promotion of tendon and tendon-bone junction repair has been reported in animal studies. Modulation of the tendon stem cell niche in vivo has also been reported to influence tendon structure. There is a need to have specific and reliable markers that can define...
journal_title:Stem cell research & therapy
pub_type: 杂志文章,评审
doi:10.1186/s13287-015-0097-y
更新日期:2015-06-02 00:00:00
abstract:BACKGROUND:Human embryonic stem cells (hESCs) hold tremendous promise for cell replacement therapies for a range of degenerative diseases. In order to provide cost-effective treatments affordable by public health systems, HLA-matched allogeneic tissue banks of the highest quality clinical-grade hESCs will be required. ...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-017-0561-y
更新日期:2017-06-05 00:00:00
abstract:BACKGROUND:Some children who have survived cancer will be azoospermic in the future. Performing isolation and purification procedures for spermatogonial stem cells (SSC) is very critical. In this regard, performing the process of decontamination of cancerous cells is the initial step. The major objective of the present...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-01671-1
更新日期:2020-05-24 00:00:00
abstract::The loss of neurons and degeneration of axons after spinal cord injury result in the loss of sensory and motor functions. A bridging biomaterial construct that allows the axons to grow through has been investigated for the repair of injured spinal cord. Due to the hostility of the microenvironment in the lesion, multi...
journal_title:Stem cell research & therapy
pub_type: 杂志文章,评审
doi:10.1186/scrt480
更新日期:2014-08-01 00:00:00
abstract:BACKGROUND:Degeneration of smooth muscles in sphincters can cause debilitating diseases such as fecal incontinence. Skeletal muscle-derived cells have been effectively used in clinics for the regeneration of the skeletal muscle sphincters, such as the external anal or urinary sphincter. However, little is known about t...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-01749-w
更新日期:2020-06-12 00:00:00
abstract:INTRODUCTION:Studies have shown that stem cells exert their therapeutic effects on acute kidney injury (AKI) through paracrine/endocrine actions. If the protective effect is mediated in an endocrine manner, the injection of the factors that these cells secrete could be effective, but the effect of conditioned medium (C...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/scrt489
更新日期:2014-08-21 00:00:00
abstract:BACKGROUND:Autologous cellular immunotherapy or immune enhancement therapy has demonstrated some promising benefits for prostate cancer. T cell-based immunotherapy or sipuleucel-T therapy has yielded certain beneficial responses and a slight improvement on the overall survival of patients with metastatic castration-res...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-01634-6
更新日期:2020-03-17 00:00:00
abstract:BACKGROUND:Mesenchymal stem cells derived from human umbilical cord tissue, termed UCX®, have the potential to promote a full range of events leading to tissue regeneration and homeostasis. The main goal of this work was to investigate UCX® action in experimentally induced hindlimb ischemia (HLI). METHODS:UCX®, obtain...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-016-0410-4
更新日期:2016-09-29 00:00:00
abstract:BACKGROUND:Lung cancer stem cells have the ability to self-renew and are resistant to conventional chemotherapy. MicroRNAs (miRNAs) regulate and control the expression and function of many target genes; therefore, miRNA disorders are involved in the pathogenesis of human diseases, such as cancer. However, the effects o...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-018-1072-1
更新日期:2018-11-23 00:00:00
abstract:INTRODUCTION:Human multipotent stromal cells (MSCs) isolated from bone marrow or other tissue sources have great potential to treat a wide range of injuries and disorders in the field of regenerative medicine and tissue engineering. In particular, MSCs have inherent characteristics to suppress the immune system and are...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/scrt448
更新日期:2014-04-28 00:00:00
abstract:BACKGROUND:Bone defects are a common clinical condition that has gained an increasing amount of attention in recent years. Causes of bone defect include tumors, inflammation, and fractures. Bone tissue engineering is a novel treatment of bone defect, and human mesenchymal stem cells (hMSCs) are the ideal seed cells for...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-01631-9
更新日期:2020-03-25 00:00:00
abstract:BACKGROUND:We aimed to generate a bioengineered multi-lamellar human corneal stroma tissue in vitro by differentiating periodontal ligament stem cells (PDLSCs) towards keratocytes on an aligned silk membrane. METHODS:Human PDLSCs were isolated and identified. The neuropeptide substance P (SP) was added in keratocyte d...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-017-0715-y
更新日期:2017-11-13 00:00:00
abstract:OBJECTIVES:Mesenchymal stem cells (MSCs) have been intensively investigated as to their therapeutic potentials. However, the full chemical-defined medium supporting the isolation and expansion of human MSCs has not been developed yet. MATERIALS AND METHODS:Here, we developed the full chemical-defined medium, NBVbe med...
journal_title:Stem cell research & therapy
pub_type: 杂志文章
doi:10.1186/s13287-020-01641-7
更新日期:2020-03-19 00:00:00