Abstract：
:Autosomal recessive Stargardt disease is the most common cause of inherited retinal disease. In this report, we describe the generation and characterization of two human induced pluripotent stem cell (iPSC) lines from a patient with compound heterozygous mutations in the ABCA4 gene (c.[768G>T];[6079C>T]). Patient dermal fibroblasts were reprogrammed using episomal plasmids encoding OCT4, SOX2, KLF4, L-MYC, LIN28, mir302/367 microRNA and shRNA for P53. The clonal iPSC lines LEIi012-A and LEIi012-B were established. Both lines had a normal karyotype, displayed iPSC morphology, expressed pluripotency genes at similar levels to control iPSC and displayed trilineage differentiation potential during embryoid body differentiation.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Jennings L,Zhang D,Chen SC,Moon SY,Lamey T,Thompson JA,McLaren T,De Roach JN,Chen FK,McLenachan Sdoi
10.1016/j.scr.2020.101947subject
Has Abstractpub_date
2020-10-01 00:00:00pages
101947eissn
1873-5061issn
1876-7753pii
S1873-5061(20)30248-8journal_volume
48pub_type
杂志文章abstract：:The KCL018 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation affecting one allele of the DMPK gene encoding the dystrophia myotonica protein kinase (2200 trinucleotide repeats; 14 for the normal allele). The ICM was isolated using laser microsurg...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.01.004
更新日期：2016-03-01 00:00:00
abstract：:The discovery of the Ten-Eleven Translocation (TET) protein family was initiated by the identification of the MLL partner TET1, and of mutations in the TET2 gene in hematological malignancies including myeloproliferative neoplasms (MPN). TET1, 2 and 3 proteins hydroxylate 5-methylcytosine (5-mC) into 5-hydroxymethylcy...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101755
更新日期：2020-04-01 00:00:00
abstract：:SLC26A4 is the second most frequent gene implicated in congenital hearing loss after GJB2 mutations. Here, we report the generation of induced pluripotent stem cells (iPSCs), from a patient who was carrying a homozygous c.919-2A>G variant in the SLC26A4 gene. This is the most common variant of SLC26A4 gene in the Chin...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101524
更新日期：2019-10-01 00:00:00
abstract：:Extracellular signal-regulated kinases (ERKs) have many important functions during embryogenesis. However, their role in embryonic stem (ES) cells is controversial. Previous studies reported that, in contrast to mouse ES cells, human ES cells differentiate if ERK1/2 is inhibited. We reexamined the role of ERK1/2 in hu...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.06.002
更新日期：2010-09-01 00:00:00
abstract：:The purpose of this study was to isolate cancer stem cells (CSCs, also called tumor-initiating cells, TICs) from established human colorectal carcinoma (CRC) cell lines, characterize them extensively and dissect the mechanism for their stemness. Freshly isolated CD44(+) and CD44(-) cells from the HCT-15 human colon ca...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.09.011
更新日期：2014-01-01 00:00:00
abstract：:The conventional method of culturing human embryonic stem cells (hESC) is on two-dimensional (2D) surfaces, which is not amenable for scale up to therapeutic quantities in bioreactors. We have developed a facile and robust method for maintaining undifferentiated hESC in three-dimensional (3D) suspension cultures on ma...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2009.02.005
更新日期：2009-05-01 00:00:00
abstract：OBJECTIVE:Fecal incontinence reduces the quality of life of many women but has no long-term cure. Research on mesenchymal stem cell (MSC)-based therapies has shown promising results. The primary aim of this study was to evaluate functional recovery after treatment with MSCs in two animal models of anal sphincter injury...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2012.10.002
更新日期：2013-01-01 00:00:00
abstract：:Tumor protein p63 (p63) encodes for a transcription factor of the p53 family and is a marker for respiratory basal cells. Based on a NKX2.1 knock-in reporter cell line from human induced pluripotent stem cells (hiPSCs) (MHHi06-A-2) we established a NKX2.1/p63 double transgenic knock-in reporter cell line using TALEN t...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101659
更新日期：2020-01-01 00:00:00
abstract：:Aside from its role in cell membrane integrity, cholesterol is a key component in steroid hormone production. The vital functions of steroid hormones such as estrogen, testosterone, glucocorticoids (Gcrts) and mineralocorticoids (Mnrts) in perinatal and adult life are well understood; however, their role during early ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.04.010
更新日期：2015-07-01 00:00:00
abstract：:In this study, we established induced pluripotent stem (iPS) cell lines from postmortem dura-derived fibroblasts of four control individuals with low polygenic risk score for psychiatric disorders including schizophrenia and bipolar disorder. The fibroblasts were reprogrammed into iPS cells using episomal vectors carr...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101806
更新日期：2020-07-01 00:00:00
abstract：:Neural progenitor cells (NPCs) are promising candidates for cell-based therapy of neurodegenerative diseases; however, safety concerns must be addressed through transplantation studies in large animal models, such as the pig. The aim of this study was to derive NPCs from porcine blastocysts and evaluate their in-vitro...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2011.04.004
更新日期：2011-09-01 00:00:00
abstract：:An 83-year old Alzheimer's disease (AD) male patient donated his Peripheral blood mononuclear cells (PBMC). The non-integrating episomal vector system used to reprogram PBMCs with the human OKSM transcription factors. The pluripotency of transgene-free iPSCs was confirmed by immunocytochemistry for pluripotency marker...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.11.008
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：:Cardiomyocyte proliferation and progenitor differentiation are endogenous mechanisms of myocardial development. Cardiomyocytes continue to proliferate in mammals for part of post-natal development. In adult mammals under homeostatic conditions, cardiomyocytes proliferate at an extremely low rate. Because the mechanism...
journal_title：Stem cell research
pub_type： 杂志文章,评审
doi：10.1016/j.scr.2014.09.003
更新日期：2014-11-01 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：:Satellite cells (SCs) are the resident stem cells of skeletal muscle tissue which play a major role in muscle adaptation, e.g. as a response to physical training. The aim of this study was to examine the effects of an intermittent lactate (La) treatment on the proliferation and differentiation of C2C12 myoblasts, simu...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2014.03.004
更新日期：2014-05-01 00:00:00
abstract：:Oligodendrocyte precursor cells (OPCs) differentiation from multipotent neural stem cells (NSCs) into mature oligodendrocytes is driven by thyroid hormone and mediated by thyroid hormone receptors (TRs). We show that several nuclear receptors display strong changes in expression levels between fetal and adult NSCs, wi...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101443
更新日期：2019-05-01 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：:The outcomes of clinical trials using bone marrow stromal cell (BMSC) are variable; the degree of the expansion of BMSCs during clinical manufacturing may contribute to this variability since cell expansion is limited by senescence. Human BMSCs from aspirates of healthy subjects were subcultured serially until cell gr...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.07.005
更新日期：2013-11-01 00:00:00
abstract：:Induced pluripotent stem cells (iPSCs) were generated from peripheral blood mononuclear cells (PBMCs) obtained from a 60-year-old female diagnosed with sporadic amyotrophic lateral sclerosis (sALS). The iPSCs shared the same karyotype with the parent PBMCs, expressed pluripotency stem cell markers, and demonstrated tr...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101841
更新日期：2020-05-01 00:00:00
abstract：:By means of retroviral transduction using the four Yamanaka-factors OCT4, SOX2, KLF4 and c-MYC primary human amniotic fluid cells (AFCs) were reprogrammed into several iPSC lines. Pluripotency was confirmed both in vitro and in vivo. A comparative transcriptome analysis of the AF-derived iPSC line 41 and the human emb...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.11.003
更新日期：2015-11-01 00:00:00
abstract：:Hypoxia affects many physiologic processes during early stages of mammalian ontogeny, particularly placental and vascular development. In the adult, the hypoxic bone marrow microenvironment plays a role in regulating hematopoietic stem cell (HSC) function. HSCs are generated from the major vasculature of the embryo, b...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.09.006
更新日期：2014-01-01 00:00:00
abstract：:Best's disease (BD) is an inherited retinal degenerative disease caused by mutations in BEST1 gene. A human induced pluripotent stem cell (iPSC) line has been generated with integration-free Sendai virus method from peripheral blood mononuclear cells (PBMCs) of a BD patient carrying c.888C > A mutation in BEST1 gene. ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101459
更新日期：2019-07-01 00:00:00
abstract：:The human iPSC line LEIi006-A was generated from dermal fibroblasts from a patient with retinitis pigmentosa using episomal plasmids containing OCT4, SOX2, KLF4, L-MYC, LIN28, mir302/367 microRNA and shRNA for p53. The iPSC cells carry compound heterozygous mutations (c.1892A > G and c.2548G > A) in the CRB1 gene. LEI...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.08.001
更新日期：2018-08-01 00:00:00
abstract：:ATOX1 is a copper chaperone involved in intracellular copper homeostasis, cell proliferation, and tumor progression. To investigate the physiologically relevant molecular mechanism of ATOX1 by using imaging-based approaches, we genetically modified ATOX1 in H1 hESCs to express mCherry-ATOX1 fusion protein under endoge...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101631
更新日期：2019-12-01 00:00:00
abstract：:FOS is component of the AP-1 complex and has been reported to be involved in many cellular functions, including cell proliferation, differentiation, survival, angiogenesis, hematopoiesis and cancer progress. To further understand the exact role of FOS in these processes, here we created two FOS knockout human embryoni...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101479
更新日期：2019-08-01 00:00:00
abstract：:The niche concept of stem cell biology proposes a functional unit between the precursor cells and their local microenvironment, to which several cell types might contribute by cell-cell contacts, extracellular matrix, and humoral factors. We here established three co-culture models (with cell types separated by membra...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.09.010
更新日期：2015-11-01 00:00:00
abstract：:We have generated a human induced pluripotent stem cell (iPSC) line derived from urinary cells of a 10years old patient with primary congenital glaucoma (PCG). The cells were reprogrammed with the human OSKM transcription factors using the Sendai-virus delivery system and shown to have full differentiation potential. ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.04.004
更新日期：2018-05-01 00:00:00
abstract：:Pluripotency is a cellular state of multiple options. Here, we highlight the potential for self-organization to contribute to stem cell fate computation. A new way of considering regulatory circuitry is presented that describes the expression of each transcription factor (TF) as a branching process that propagates thr...
journal_title：Stem cell research
pub_type： 杂志文章,评审
doi：10.1016/j.scr.2011.11.001
更新日期：2012-03-01 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
