Abstract：
:Biliary atresia (BA) is a common cause of pediatric end-stage liver disease. While its etiology is not yet clear, evidence has suggested that BA results from interactions between genetic susceptibility and environmental factors. Disease relevant human cellular models of BA will facilitate identification of both genetic and environmental factors that are important for disease prevention and treatment. Here we report the generation of a human induced pluripotent stem cell line from a BA patient using episomal vectors. Patient-specific BA iPSC lines provide valuable tools for disease mechanism study and drug development.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Tian L,Eldridge L,Chaudhari P,Zhang L,Anders RA,Schwarz KB,Ye Z,Jang YYdoi
10.1016/j.scr.2017.08.001subject
Has Abstractpub_date
2017-10-01 00:00:00pages
25-28eissn
1873-5061issn
1876-7753pii
S1873-5061(17)30156-3journal_volume
24pub_type
杂志文章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：:Over-expression of the early neural inducer, Noggin, in nestin positive subventricular zone (SVZ), neural stem cells (NSC) promotes proliferation and neuronal differentiation of neural progenitors and inhibits the expression of a CNS-enriched microRNA-410 (miR-410) (Morell et al., 2015). When expressed in neurospheres...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.07.003
更新日期：2016-09-01 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：:As one of the most essential genome guardians, p53 and its mutants have been suggested associated with many types of cancers. Many p53 mutants function induce unique phenotypes, including carcinogenesis, metastasis, and drug resistance. The p53(R249S) mutation is the most prevalent and specific mutation associated wit...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.101360
更新日期：2019-01-01 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：: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：:Human lymphoblast cells from a female and male patient diagnosed with Alzheimer's disease (AD) with different genotypes of a functional copy number variation (CNV) in the AD risk gene CR1 were used to generate integration-free induced pluripotent stem cells (iPSCs) employing episomal plasmids expressing OCT4, SOX2, NA...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.10.003
更新日期：2016-11-01 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：:CHCHD2 mutation has been reported as a potential cause of a rare form of familial Parkinson's disease. Recently, a novel CHCHD2 mutation was identified in a family with Parkinson's disease. The dermal fibroblasts of the patient were obtained and successfully transformed into induced pluripotent stem cells(iPSCs), empl...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.08.011
更新日期：2018-10-01 00:00:00
abstract：:A DAND5-control human iPSC line was generated from the urinary cells of a phenotypically normal donor. Exfoliated renal epithelial (RE) cells were collected and reprogrammed into iPSCs using Sendai virus reprogramming system. The pluripotency, in vitro differentiation potential, karyotype stability, and the transgene-...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.04.015
更新日期：2018-05-01 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：BACKGROUND:Non-random segregation of DNA strands during stem cell replication has been proposed as a mechanism to minimize accumulated genetic errors in stem cells of rapidly dividing tissues. According to this hypothesis, an "immortal" DNA strand is passed to the stem cell daughter and not the more differentiated cell...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.01.005
更新日期：2015-03-01 00:00:00
abstract：:Insulin-like growth factor 1 (IGF-1) plays an important role in the regulation of tooth root development, and stem cells from apical papilla (SCAPs) are responsible for the formation of root pulp and dentin. To date, it remains unclear whether IGF-1 can regulate the function of SCAPs. In this study, SCAPs were isolate...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2011.12.005
更新日期：2012-05-01 00:00:00
abstract：:We describe the generation and characterization of 5 human induced pluripotent stem cell (iPSC) lines derived from peripheral blood mononuclear cells (PBMCs) of healthy adult individuals. The PBMCs were reprogrammed using non-integrating Sendai viruses containing the reprogramming factors POU5F1 (OCT4), SOX2, KLF4 and...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.101380
更新日期：2019-01-01 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：:Maturity onset diabetes of the young (MODY) is a hereditary form of diabetes mellitus presenting at childhood or adolescence, which eventually leads to pancreatic β-cells dysfunction. The underlying genetic basis of MODY disorders is haploinsufficiency, where loss-of-function mutations in a single allele cause the dia...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.06.013
更新日期：2018-08-01 00:00:00
abstract：:Two distinct types of embryonic pluripotent stem cells can be established from either the inner cell mass (ICM) of preimplantation blastocyst (leukemia inhibitory factor (LIF)-dependent embryonic stem cell, ESC, called naive state) or the epiblast of postimplantation fetuses (fibroblast growth factor 2 (FGF2)-dependen...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2014.04.012
更新日期：2014-07-01 00:00:00
abstract：:Neural progenitor cells have been proposed as a therapy for central nervous system disorders, including neurodegenerative diseases and trauma injuries, however their accessibility is a major limitation. We recently isolated Tuj1+ cells from skeletal muscle culture of Nestin-GFP transgenic mice however whether they for...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2012.09.003
更新日期：2013-01-01 00:00:00
abstract：:The Genea016 human embryonic stem cell line was derived from a donated, fully commercially consented ART blastocyst, through ICM outgrowth on inactivated human feeders. The line showed pluripotent cell morphology and genomic analysis verified a 46, XX karyotype and female Allele pattern through traditional karyotyping...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.11.009
更新日期：2016-01-01 00:00:00
abstract：:The Mesenchymal-to-Epithelial Transition (MET) has been recognized as a crucial step for successful reprogramming of fibroblasts to induced pluripotent stem cells (iPSCs). Thus, it has been demonstrated, that the efficiency of reprogramming can be enhanced by promoting an epithelial expression program in cells, with a...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2014.11.003
更新日期：2015-01-01 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：: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：: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 derm...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101947
更新日期：2020-10-01 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：:The peptide hormone insulin produced by pancreatic β-cells undergoes post-transcriptional processing before secretion. In particular, C-peptide is cleaved from pro-insulin to generate mature insulin. Here, we introduce a C-peptide-mCherry human iPSC line (HMGUi001-A-8). The line was generated by CRISPR/Cas9 mediated h...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102126
更新日期：2020-12-16 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：: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：:Hematopoietic stem and progenitor cell (HSPC) transplantation is the paradigm for stem cell therapies. The protocol described here enables quantitative assessment of the body-wide HSPC reconstitution of different mature hematopoietic cells in mice based on their presence in circulating blood. The method determines don...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102145
更新日期：2020-12-29 00:00:00
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：:Skin fibroblasts were obtained from four patients with Williams-Beuren syndrome (WBS) carrying the typical 1.5 Mb or 1.8 Mb deletion at the 7q11.23 genomic region. Induced pluripotent stem cells (iPSCs) were generated by retroviral infection of fibroblasts with polycystronic vectors. The generated iPSC clones ESi059A,...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102087
更新日期：2020-12-01 00:00:00
