Abstract:
:Human embryonic stem (hES) cells have the dual ability to self-renew and differentiate into specialized cell types. However, in order to realize the full potential of these cells it is important to understand how the genes responsible for their unique characteristics are regulated. In this study we examine the regulation of the tropomyosin-related kinase (TRK) genes which encode for receptors important in hES cell survival and self-renewal. Although the TRK genes have been studied in many neuronal cell types, the regulation of these genes in hES cells is unclear. Our study demonstrates a novel regulatory relationship between the TRKC gene and the transcription factor SOX2. Our results found that hES cells highly express full-length and truncated forms of the TRKC gene. However, examination of the related TRKB gene showed a lower overall expression of both full-length and truncated forms. Through RNA interference, we knocked down expression levels of SOX2 in hES cells and examined the expression of TRKC, as well as TRKB. Upon loss of SOX2 we found that TRKC mRNA levels were significantly downregulated but TRKB levels remained unchanged, demonstrating an important regulatory dependence on SOX2 by TRKC. We also found that TRKC protein levels were also decreased after SOX2 knock down. Further analysis found the regulatory region of TRKC to be highly conserved among many mammals with potential SOX binding motifs. We confirmed a specific binding motif as a site that SOX2 utilizes to directly interact with the TRKC regulatory region. In addition, we found that SOX2 drives expression of the TRKC gene by activating a luciferase reporter construct containing the TRKC regulatory region and the SOX binding motif.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Fong H,Wong RC,Donovan PJdoi
10.1016/j.scr.2011.10.003subject
Has Abstractpub_date
2012-03-01 00:00:00pages
206-14issue
2eissn
1873-5061issn
1876-7753pii
S1873-5061(11)00140-1journal_volume
8pub_type
杂志文章abstract::Members of the Fanconi anemia (FA) protein family are involved in multiple cellular processes including response to DNA damage and oxidative stress. Here we show that a major FA protein, Fancd2, plays a role in mitochondrial biosynthesis through regulation of mitochondrial translation. Fancd2 interacts with Atad3 and ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2019.101550
更新日期:2019-10-01 00:00:00
abstract::The human iPSC cell line, CARS-FiPS4F1 (ESi064-A), derived from dermal fibroblast from the apparently healthy carrier of the mutation of the gene SACSIN, was generated by non-integrative reprogramming technology using OCT3/4, SOX2, CMYC and KLF4 reprogramming factors. The pluripotency was assessed by immunocytochemist...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2018.10.016
更新日期:2018-12-01 00:00:00
abstract::Oligodendrocytes, which are the main cell type in cerebral white matter, are generated from their precursor cells (oligodendrocyte precursor cells: OPCs). However, the differentiation from OPCs to oligodendrocytes is disturbed under stressed conditions. Therefore, drugs that can improve oligodendrocyte regeneration ma...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2015.05.001
更新日期:2015-07-01 00:00:00
abstract::Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by a CAG-repeat expanding mutation in ATXN3. We generated induced pluripotent stem cells (iPSCs) from a SCA3 patient by electroporation of dermal fibroblasts with episomal plasmids encoding L-MYC, LIN28, SOX2, KLF4, OCT4 an...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2016.02.040
更新日期:2016-05-01 00:00:00
abstract::Neuroblasts represent the predominant migrating cell type in the adult mouse brain. There are, however, increasing evidences of migration of other neural precursors. This work aims at identifying in vivo endogenous early neural precursors, different from neuroblasts, able to migrate in response to brain injuries. The ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2014.11.006
更新日期:2015-01-01 00:00:00
abstract::We have established the patient-specific induced pluripotent stem (iPS) cell line CSUASOi004-A by using peripheral blood mononuclear cells (PBMCs) of a retinitis pigmentosa (RP) patient with a PRPF6 gene mutation (c.G2699A:p.R900H). CSUASOi004-A was established by a non-integrative method with four episomal plasmids c...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.101793
更新日期:2020-05-01 00:00:00
abstract::Transgene free UNIPDi002-A-human induced pluripotent stem cell (hiPSC) line was generated by Sendai Virus Vectors reprogramming from human oral mucosal epithelial stem cells (hOMESCs) of a patient affected by ectrodactyly-ectodermal dysplasia-clefting (EEC)-syndrome, carrying a mutation in exon 8 of the TP63 gene (R30...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2018.02.006
更新日期:2018-04-01 00:00:00
abstract::Autism is a heterogeneous neurodevelopmental disorder defined by deficits in socialization, communication, and patterns of behavior. Using stem cells to model brain disordersmay yield new understanding about the underlying neuropathological processes and could prove essential for drug development. We present here a ne...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.102038
更新日期:2020-12-01 00:00:00
abstract::The purpose of this study is to establish a murine embryonic stem cell (mESC) line for isolation of functional ventricular cardiomyocytes (VCMs) and then to characterize the derived VCMs. By crossing the myosin light chain 2v (Mlc2v)-Cre mouse line with the reporter strain Rosa26-yellow fluorescent protein (YFP), we g...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2011.08.004
更新日期:2012-01-01 00:00:00
abstract::Cell-mediated gene therapy is a possible means to treat muscular dystrophies like Duchenne muscular dystrophy. Autologous patient stem cells can be genetically-corrected and transplanted back into the patient, without causing immunorejection problems. Regenerated muscle fibres derived from these cells will express the...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2018.05.004
更新日期:2018-07-01 00:00:00
abstract::Derivation of induced pluripotent stem (iPS) cells is mainly an epigenetic reprogramming process. It is still quite controversial how genomic imprinting is reprogrammed in iPS cells. Thus, we derived multiple iPS clones from genetically identical mouse somatic cells. We found that parentally inherited imprint was vari...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2013.05.011
更新日期:2013-09-01 00:00:00
abstract::Many studies have compared the genetic and epigenetic profiles of human induced pluripotent stem cells (hiPSCs) to human embryonic stem cells (hESCs) and yet the picture remains unclear. To address this, we derived a population of neural precursor cells (NPCs) from the H1 (WA01) hESC line and generated isogenic iPSC l...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2013.11.010
更新日期:2014-03-01 00:00:00
abstract::The human embryonic stem cell line RCe019-A (RC-15) was derived under quality assured compliance with UK regulation, European Union Directives and International guidance for tissue procurement, processing and storage according to Good Manufacturing Practice (GMP) standards. The cell line was derived from a cleavage st...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2016.04.003
更新日期:2016-05-01 00:00:00
abstract::Human teratoma is a germ cell tumor that contains normal tissues (e.g., hair, skin or cartilage) differentiated from embryonal germ layers. Because of the feature of this tumor, we hypothesized that human teratomas contain multipotent stem cells that can develop into various non-cancerous normal tissues. In this study...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2019.101633
更新日期:2019-12-01 00:00:00
abstract::We sought to elucidate how and when the ocular surface ectoderm commits to its differentiation into the corneal epithelium in eye development from human induced pluripotent stem cells (hiPSCs) under the influence of WNT signaling and the actions of BMP4. These signals are key drivers ocular surface ectodermal cell fat...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.101868
更新日期:2020-07-01 00:00:00
abstract::Recently, many hurdles and limitations for production of clinically applicable iPSC derivatives have been overcome. Transgene-free iPSCs can be efficiently derived from easily accessible cell sources such as blood. Here we describe the generation of transgene-free hiPS cells from cord blood derived CD34+ cells, reprog...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.03.022
更新日期:2017-05-01 00:00:00
abstract::Heredity is the major factor contributing to the susceptibility to ankylosing spondylitis(AS). Janus kinase 2 (JAK2) has been associated with AS. Urine-derived cells from an AS patient with JAK2 mutation were used to generate induced pluripotent stem cells (iPSCs) with five episomal iPSC reprogramming vectors (pCXLE-h...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.101788
更新日期:2020-05-01 00:00:00
abstract::There are currently no reports of identification of stem cells in human gallbladder. The differences between human gallbladder and intrahepatic bile duct (IHBD) cells have also not been explored. The goals of this study were to evaluate if human fetal gallbladder contains a candidate stem cell population and if fetal ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2014.12.003
更新日期:2015-05-01 00:00:00
abstract::Optimization of pluripotent stem cell expansion and differentiation is facilitated by biological tools that permit non-invasive and dynamic monitoring of pluripotency, and the ability to select for an undifferentiated input cell population. Here we report on the generation and characterisation of clonal human embryoni...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2014.05.006
更新日期:2014-09-01 00:00:00
abstract::Parental origin-dependent expression of the imprinted genes is essential for mammalian development. Zfp57 maintains genomic imprinting in mouse embryos and ES cells. To examine the allelic expression patterns of the imprinted genes in ES cells, we obtained multiple hybrid ES clones that were directly derived from the ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2016.01.017
更新日期:2016-03-01 00:00:00
abstract::Adult skeletal muscle regeneration relies on the activity of satellite cells residing in the skeletal muscle niche. However, systemic and intrinsic factors decrease the myogenic differentiation potential of satellite cells thereby impairing muscle regeneration. Here we present data showing that late passage C2C12 myob...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.11.018
更新日期:2018-01-01 00:00:00
abstract::Asparagine synthetase (ASNS) deficiency (ASNSD; MIM #615574) is a rare neurodevelopmental disorder caused by mutations in the ASNS gene. The ASNS gene maps to cytogenetic band 7q21.3 and is 35 kb long. ASNSD is characterised by congenital microcephaly, severely delayed psychomotor development, seizures, and hyperekple...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2019.101583
更新日期:2019-12-01 00:00:00
abstract::Hypertrophic cardiomyopathy is an inherited cardiomyopathy with a prevalence of up to 1 in 200, which can result in significant morbidity and mortality. An iPSC line was generated from peripheral blood mononuclear cells obtained from the whole blood of a 58-year-old male with hypertrophic cardiomyopathy who carries th...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2018.10.009
更新日期:2018-12-01 00:00:00
abstract::Umbilical cord blood (UCB) transplantation has emerged as a promising therapy, but it is challenged by scarcity of stem cells. Eltrombopag is a non-peptide, thrombopoietin (TPO) receptor agonist, which selectively activates c-Mpl in humans and chimpanzees. We investigated eltrombopag's effects on human UCB hematopoiet...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2012.05.001
更新日期:2012-09-01 00:00:00
abstract::We report here the generation of human iPS cell line UKKi009-A from dermal fibroblasts of a patient carrying heterozygous mutation c.3035-3045delTCCCTCGATGC, p.Leu1012Pro (fs*55) in KCNH2 gene leading to long QT syndrome type 2 (LQT2). We used the Sleeping Beauty transposon-based plasmids expressing OSKM along with mi...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2015.12.039
更新日期:2016-03-01 00:00:00
abstract::Human iPSC line N44SV.5 was generated from primary normal human dermal fibroblasts belonging to the European mitochondrial haplogroup U. For this purpose, reprogramming factors Oct3/4, Sox2, Klf4, and cMyc were delivered using a non-integrative methodology that involves the use of Sendai virus. ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2015.12.010
更新日期:2016-01-01 00:00:00
abstract::We generated human induced pluripotent stem cells (hiPSCs) from dermal fibroblasts of a 51years old female patient homozygous for the mutation c.535 G>A p.G179S on the KCNQ1 gene, causing a severe form of autosomal recessive Long QT Syndrome type 1 (AR-LQT1), not associated with deafness. The hiPSCs, generated using f...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2018.04.003
更新日期:2018-05-01 00:00:00
abstract::Gene therapy mediated by bone marrow-derived hematopoietic stem cells (BM-HSC) has been widely used in treating genetic deficiencies in both pre-clinical and clinical settings. Using mitotically inactive cell-targeting lentivirus with separate promoters for our gene of interest (the murine MHC class II (MHCII) chapero...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2013.10.010
更新日期:2014-01-01 00:00:00
abstract::Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder characterized by a genetic predisposition for thoracic aortic aneurysm and dissection. Despite heterozygous loss-of-function mutations in genes for ligand, receptor, or downstream mediators of the transforming growth factor β (TGFβ) pathway, LDS is associ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.101707
更新日期:2020-03-01 00:00:00
abstract::Human iPSC line was generated from patient-specific adipose tissue-derived mesenchymal multipotent stromal cells carrying two mutations in plakophilin-2 (PKP2) gene using non-integrative reprogramming method. Reprogramming factors OCT4, KLF4, SOX2, CMYC were delivered using Sendai viruses. Pluripotency was confirmed i...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.08.014
更新日期:2017-10-01 00:00:00