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 contains a 24-nucleotide deletion within exon-13 of GDE, resulting in 8 amino acids (TRLGISSL) missing of the GDE protein from amino acid position 567 to 575. The WAe001-A-14 cell line maintains typical stem cell morphology, pluripotency and in vitro differentiation potential, and a normal karyotype.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Xu G,Guo D,Wu F,Abbas N,Lai K,Yuan F,You K,Liu Y,Zhuang Y,Wu Y,Xu Y,Chen Y,Yang F,Pan T,Li YXdoi
10.1016/j.scr.2017.12.009subject
Has Abstractpub_date
2018-03-01 00:00:00pages
38-41eissn
1873-5061issn
1876-7753pii
S1873-5061(17)30267-2journal_volume
27pub_type
杂志文章abstract：:Gastrointestinal (GI) homeostasis requires the action of multiple pathways. There is some controversy regarding whether small intestine (SI) Paneth cells (PCs) play a central role in orchestrating crypt architecture and their relationship with Lgr5+ve stem cells. Nevertheless, we previously showed that germline CSF-1 ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2012.12.001
更新日期：2013-03-01 00:00:00
abstract：:In the present study, we investigated the effect of simultaneous downregulation of uPAR and cathepsin B (pUC), alone or in combination with radiation, on JNK-MAPK signaling pathway in regulating the migration of non-GICs (glioma-initiating cells) and GICs. The increase in the expression of p-JNK with pUC treatment was...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2014.02.008
更新日期：2014-05-01 00:00:00
abstract：:Salisphere-derived adult epithelial cells have been used to improve saliva production of irradiated mouse salivary glands. Importantly, optimization of the cellular composition of salispheres could improve their regenerative capabilities. The Rho Kinase (ROCK) inhibitor, Y27632, has been used to increase the prolifera...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101608
更新日期：2019-12-01 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：: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：:Interactions between acute myeloid leukemia (AML) blasts and neighboring stromal cells are important for disease development and chemosensitivity. However, the molecular mechanisms involved in the cytokine-mediated crosstalk between mesenchymal stem cells (MSCs) and AML cells are largely unknown. Leukemic cells derive...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.09.008
更新日期：2015-11-01 00:00:00
abstract：:A recessive mutation in PLA2G6, which is known to cause a heterogeneous neurodegenerative clinical spectrum, has recently been shown to be responsible for autosomal-recessive familial forms of Parkinson's disease (PD). Here, we generated induced pluripotent stem cells (iPSCs) from the peripheral blood mononuclear cell...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101432
更新日期：2019-05-01 00:00:00
abstract：:Human induced pluripotent stem cell-lines (iPSCs) of Indian origin NCCSi005A and NCCSi006A were established by reprogramming of CD4+T cells, isolated from the peripheral blood mononuclear cells (PBMCs) of two healthy female donors. Reprogramming was achieved using integration free, Sendai viral vector system expressin...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101506
更新日期：2019-08-01 00:00:00
abstract：:Mouse embryonic stem cell (mESC) lines were derived by crossing heterozygous transgenic (tg) mice expressing green fluorescent protein (GFP) under the control of the rat tyrosine hydroxylase (TH) promoter, with homozygous alpha-synuclein (aSYN) mice expressing human mutant SNCAA53T under the control of the mouse Prion...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.12.026
更新日期：2017-03-01 00:00:00
abstract：:The MB49 bladder cancer cell vaccine was effective against bladder cancer in the mice model in previous studies. However, part of the tumors regrew as the vaccine could not eliminate the cancer stem cells (CSCs). MB49 bladder cancer stem cells (MCSCs) were isolated by a combination of the limited dilution method and t...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2014.04.006
更新日期：2014-07-01 00:00:00
abstract：:Bone marrow stromal cell (BMSC) adhesion and migration are fundamental to a number of pathophysiologic processes, including fracture and wound healing. Vitamin C is beneficial for bone formation, fracture repair and wound healing. However, the role of the vitamin C transporter in BMSC adhesion, migration and wound hea...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.11.002
更新日期：2014-03-01 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：:Generating human podocytes in vitro could offer a unique opportunity to study human diseases. Here, we describe a simple and efficient protocol for obtaining functional podocytes in vitro from human induced pluripotent stem cells. Cells were exposed to a three-step protocol, which induced their differentiation into in...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.06.001
更新日期：2016-07-01 00:00:00
abstract：:The human transcription factor NKX2-5 plays an important role in cardiac formation and development, and thus it can be used for isolation of cardiomyocytes (CMs) differentiated from human pluripotent stem cells (hPSCs). Here, we knocked-in enhanced GFP (eGFP) and Pac (a puromycin resistant gene; PuroR) into the exon 1...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102120
更新日期：2020-12-10 00:00:00
abstract：:Understanding the complex mechanisms that govern the fate decisions of human embryonic stem cells (hESCs) is fundamental to their use in cell replacement therapies. The progress of dissecting these mechanisms will be facilitated by the availability of robust high-throughput screening assays on hESCs. In this study, we...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.04.006
更新日期：2010-09-01 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：: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：: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：:Pathological myopia (PM) is a retinal degenerative disease with an increasing prevalence in Asia. The peripheral blood mononuclear cells (PBMCs) from a patient with PM were successfully reprogrammed to induced pluripotent stem cells (iPSCs) using integration-free method, Sendai viral (SeV) vectors expressing OCT4, SOX...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.101369
更新日期：2019-01-01 00:00:00
abstract：:Human embryonic stem cells (hESCs) can undergo unlimited self-renewal and differentiate into hepatic cells, including expandable hepato-blasts (HBs) and hepatocyte-like cells (HLCs) in vitro. Therefore, hESC-derived HBs have the potential to become a renewable cell source for cell therapy of serious liver damage. Howe...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102010
更新日期：2020-12-01 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：:OCT4 and NANOG are core transcription factor genes in self-renewal, differentiation, and reprogramming. Here, we generated an OCT4-EGFP, NANOG-tdTomato dual reporter hiPSC line, KKUi001-A, on the basis of human induced pluripotent stem cells using CRISPR/Cas9 technology. EGFP and tdTomato reporter were inserted into b...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101943
更新日期：2020-10-01 00:00:00
abstract：:Characterisation of mouse pluripotent stem cells has revealed two distinct pluripotent states, naive and primed, that maintain characteristics of the pre and post implanted epiblast respectively. Recent studies have developed several culture systems that seek to recapitulate the naive phenomenon in human pluripotent s...
journal_title：Stem cell research
pub_type： 杂志文章,评审
doi：10.1016/j.scr.2017.11.017
更新日期：2018-01-01 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：:The CR-4 human embryonic stem cell line was derived from the inner cell mass of a developing blastocyst. This cell line has been adapted to grow in feeder-free conditions and is especially well-suited for differentiation to retinal pigment epithelium. The line demonstrates a normal human 46,XX female karyotype. Plurip...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.12.005
更新日期：2017-01-01 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：: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：:Induced-pluripotent stem cells (iPSCs) are a potential alternative cell source in regenerative medicine, which includes the use of differentiated iPSCs for cell therapies to treat coronary artery and/or peripheral arterial diseases. Late-outgrowth endothelial progenitor cells (late-EPCs) are a unique primary cell pres...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2012.11.006
更新日期：2013-03-01 00:00:00
abstract：:Peripheral blood mononuclear cells (PBMCs) were collected from a 6-year-old female child who was clinically diagnosed as primary nephrotic syndrome (NS) with hormone resistance. An iPSC line was successfully established by the Sendai-virus (SeV) delivery system. The iPS-19 (GSPHi001-A) expressed pluripotent markers, e...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101661
更新日期：2019-12-01 00:00:00
