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. LEIi006-A expressed pluripotent stem cell markers, had a normal karyotype and could be differentiated into endoderm, mesoderm and ectodermal lineages, as well as retinal organoids.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Zhang X,Zhang D,Chen SC,Lamey T,Thompson JA,McLaren T,De Roach JN,Chen FK,McLenachan Sdoi
10.1016/j.scr.2018.08.001subject
Has Abstractpub_date
2018-08-01 00:00:00pages
147-151eissn
1873-5061issn
1876-7753pii
S1873-5061(18)30193-4journal_volume
31pub_type
杂志文章abstract::BRCA1 germline mutation confers hereditary predisposition for breast and ovarian cancer. To understand the physiopathology of mammary and ovarian epithelial cancer transformation, and to identify early driver molecular events, we have generated an iPSC line from a patient carrying a germline exon 17 deletion in BRCA1 ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.09.003
更新日期:2017-10-01 00:00:00
abstract::Our work aimed at evaluating the role of adipose stem cells (ASC) on chondrocytes from osteoarthritic (OA) patients and identifying the mediators involved. We used primary chondrocytes, ASCs from different sources and bone marrow mesenchymal stromal cells (MSC) from OA donors. ASCs or MSCs were co-cultured with chondr...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2013.05.008
更新日期:2013-09-01 00:00:00
abstract::X-linked Alport syndrome (XLAS) is a rare form of hereditary nephritis caused by mutations in the COL4A5 gene encoding the type IV collagen α5 chain. A skin biopsy was performed on one female patient with XLAS who carried a heterozygous p.G409S (c. 1225 G > A) mutation in the COL4A5 gene. A human-induced pluripotent s...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.101833
更新日期:2020-05-01 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::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::Botulinum neurotoxins (BoNTs) inhibit cholinergic synaptic transmission by specifically cleaving proteins that are crucial for neurotransmitter exocytosis. Due to the lethality of these toxins, there are elevated concerns regarding their possible use as bioterrorism agents. Moreover, their widespread use for cosmetic ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2011.01.002
更新日期:2011-05-01 00:00:00
abstract::SOX4 has been shown to promote neuronal differentiation both in the adult and embryonic neural progenitors. Ectopic SOX4 expression has also been shown to inhibit oligodendrocyte differentiation in mice, however the underlying molecular mechanisms remain poorly understood. Here we demonstrate that SOX4 regulates trans...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2018.10.005
更新日期:2018-12-01 00:00:00
abstract::The KCL037 human embryonic stem cell line was derived from a normal healthy blastocyst donated for research. The ICM was isolated using laser microsurgery and plated on γ-irradiated human foreskin fibroblasts. Both the derivation and cell line propagation were performed in an animal product-free environment and under ...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2015.12.023
更新日期:2016-01-01 00:00:00
abstract::Urine resource cells were collected from a 23-year-old male with multiple endocrine neoplasia type 1 syndrome (MEN1) for generating iPS cells with episomal plasmids. Two stable iPSC lines with free of episomal plasmid were established. The patient has a heterozygous G>T mutation on the exon 9 of Men1 gene that was con...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2016.12.002
更新日期:2017-01-01 00:00:00
abstract::Both BMP and Wnt signaling control stem cells in bulge/dermal papilla, intestinal crypt, and bone marrow. To explore their roles in the limbal niche, which govern corneal epithelial homeostasis, we established an in vitro model of sphere growth by reunion between single limbal epithelial progenitor cells (LEPCs) and a...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2014.01.003
更新日期:2014-03-01 00:00:00
abstract::Reprogramming technology enables the production of neural progenitor cells (NPCs) from somatic cells by direct transdifferentiation. However, little is known on how neural programs in these induced neural stem cells (iNSCs) differ from those of alternative stem cell populations in vitro and in vivo. Here, we performed...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2016.04.015
更新日期:2016-05-01 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::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::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::The Global Alliance for iPSC Therapies (GAiT) is a new initiative to support the implementation and clinical application of therapies derived from pluripotent stem cells to the benefit of patients globally. GAiT's mission is to serve as a central, international resource for those organisations developing therapies fro...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.102036
更新日期:2020-12-01 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::Haploidentical hematopoietic stem-cell transplantation (haplo-HSCT) is associated with an increased risk of graft failure and severe graft-versus-host disease (GVHD). Mesenchymal stromal cells (MSCs) have been shown to support in vivo normal hematopoiesis and to display potent immunesuppressive effects. We cotransplan...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2013.10.001
更新日期:2014-01-01 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::Glycogen debranching enzyme (GDE) plays a critical role in glycogenolysis. Mutations in the GDE gene are associated with a metabolic disease known as glycogen storage disease type III (GSDIII). We generated a mutant GDE human embryonic stem cell line, WAe001-A-14, using the CRISPR/Cas9 editing system. This cell line c...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.12.009
更新日期:2018-03-01 00:00:00
abstract::Neurogenesis has been shown to occur in the cerebral cortex in adult rats after ischemic stroke. The origin of the newborn neurons is largely unknown. This study aimed to explore cell division in the poststroke penumbral cortex. Adult male Wistar rats were subjected to photothrombotic ring stroke. After repeated deliv...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2008.07.003
更新日期:2009-01-01 00:00:00
abstract::Niemann-Pick disease type A (NPA) is a rare autosomal recessive lysosomal storage disease caused by mutations in the SMPD1 gene, which encodes for the protein acid sphingomyelinase. A human induced pluripotent stem cell (iPSC) line was generated from dermal fibroblasts of a 21-fetal-week-old female patient with NPA th...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2019.101461
更新日期:2019-07-01 00:00:00
abstract::The cancer stem cell model postulates that tumors are hierarchically organized with a minor population, the cancer stem cells, exhibiting unlimited proliferative potential. These cells give rise to the bulk of tumor cells, which retain a limited ability to divide. Without successful targeting of cancer stem cells, tum...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.11.009
更新日期:2018-01-01 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::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::Laminins are one of the major protein groups in the extracellular matrix (ECM) and specific laminin isoforms are crucial for neuronal functions in the central nervous system in vivo. In the present study, we compared recombinant human laminin isoforms (LN211, LN332, LN411, LN511, and LN521) and laminin isoform fragmen...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2017.09.002
更新日期:2017-10-01 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::The European Bank for induced Pluripotent Stem Cells (EBiSC), a non-profit repository for storage, banking, Quality Control (QC) and subsequent distribution of research-grade human induced Pluripotent Stem Cell (iPSC) lines, has centralised iPSC lines generated internationally across >35 disease areas and made them av...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2020.102034
更新日期:2020-12-01 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::To develop a disease model for the human 'brittle bone' disease, osteogenesis imperfecta, we have used gene editing to produce a facsimile of the patient heterozygous COL1A1 mutation in an established control iPSC line. The gene-edited line had a normal karyotype, expressed pluripotency markers and differentiated into...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2019.101449
更新日期:2019-05-01 00:00:00
abstract::Large-scale production of human induced pluripotent stem cells (hiPSCs) by robust and economic methods has been one of the major challenges for translational realization of hiPSC technology. Here we demonstrate a scalable culture system for hiPSC expansion using the E8 chemically defined and xeno-free medium under eit...
journal_title:Stem cell research
pub_type: 杂志文章
doi:10.1016/j.scr.2013.07.011
更新日期:2013-11-01 00:00:00