Abstract：
:Induced pluripotent stem cells (iPSCs) were generated from peripheral blood mononuclear cells (PBMCs) isolated from the peripheral blood of a five months-old boy with glycogen storage disease type II(GSD II, also known as Pompe disease, PD) carries compound mutations R608X E888X in GAA gene. PBMCs were reprogrammed using non-integrative Sendai viral vectors containing reprogramming factors OCT4, SOX2, KLF4 and C-MYC. iPSCs were shown to express pluripotent markers, have trilineage differentiation potential, carry GAA-R608X and GAA-E888X compound mutations, have a normal karyotype. It is useful tool for studying GSDII.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Zhang Y,Li A,Wang J,Wang G,Wang Ddoi
10.1016/j.scr.2019.101621subject
Has Abstractpub_date
2019-12-01 00:00:00pages
101621eissn
1873-5061issn
1876-7753pii
S1873-5061(19)30251-Xjournal_volume
41pub_type
杂志文章abstract：:Mesenchymal stem cells (MSC) have been isolated from almost every adult tissue. In cord blood (CB), different non-hematopoietic CD45-, CD34- adherent cell populations can be generated: the cord blood derived MSC (CB-MSC), that behave almost like MSC from bone marrow (BM-MSC), and unrestricted somatic stem cells (USSC)...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.03.004
更新日期：2010-07-01 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：:OCT4 is a highly conserved gene and plays an important role during early embryonic development and differentiation. Similar to human OCT4, mouse Oct4 gene generates variants. Oct4A is a master regulator of self-renewal in pluripotent stem cells. In this study, we have identified a novel Oct4 spliced variant, designate...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2012.04.004
更新日期：2012-09-01 00:00:00
abstract：:Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by a CAG repeat expansion in the region of the ATXN3 gene. The main feature of SCA3 is progressive ataxia, which affects balance, gait, and speech. Urine cells (UCs) of a SCA3 patient were successfully translated to induced...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.01.027
更新日期：2018-04-01 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：: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：:Transplantation of primary hepatocytes has been used in treatments for various liver pathologies and end-stage liver disease. However, shortage of donor tissue and the inability of hepatocyte proliferation in vitro have lead to alternative methods such as stem cell-derived hepatocyte-like cells (HLCs). Mesenchymal str...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101471
更新日期：2019-07-01 00:00:00
abstract：:We studied the susceptibility of human embryonic stem cells and derived contractile embryoid bodies from WAO9, HUES-5 and HUES-16 cell lines to Coxsackievirus B infection. After validating stem cell-like properties and cardiac phenotype, Coxsackievirus B receptors CAR and DAF, as well as type I interferon receptors we...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.09.002
更新日期：2011-01-01 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：: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：:Little is known about the functions of downstream regulatory element antagonist modulator (DREAM) in embryonic stem cells (ESCs). However, DREAM interacts with cAMP response element-binding protein (CREB) in a Ca(2+)-dependent manner, preventing CREB binding protein (CBP) recruitment. Furthermore, CREB and CBP are inv...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.03.001
更新日期：2016-05-01 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：:A skin biopsy was obtained from a 14-year-old female patient with a history of Myelomeningocele. Dermal fibroblasts were isolated and reprogrammed with Sendai virus (SeV) vectors encoding OCT3/4, SOX2, KLF4, and c-MYC. The generated induced Pluripotent Stem Cell (iPSC) clones NTDi4_09A were free of genomically integra...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.05.007
更新日期：2018-08-01 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：:Progenitor cells in the adult pancreas are potential sources of endocrine beta cells for treating type 1 diabetes. Previously, we identified tri-potent progenitor cells in the adult (2-4month-old) murine pancreas that were capable of self-renewal and differentiation into duct, acinar, and endocrine cells in vitro. The...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.11.015
更新日期：2016-01-01 00:00:00
abstract：:Novel and complementary experimental models are required for investigating the molecular mechanisms underlying the resistance to the available therapies of patients with major depression (Treatment-Resistant Depression, TRD) that occurs in at least one third of patients and need to be deeply investigated. Here, we hav...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102104
更新日期：2020-12-01 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：: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：:Measurement of Alkaline Phosphatase (ALP) level is a widely used procedure in clinical and basic research. We present a simple and inexpensive luminescence-based method that allows multiplexed measurement and normalization of intracellular ALP levels in one sample well. The method comprises two commercially available ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2017.07.014
更新日期：2017-08-01 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：:Fanconi anemia is a genetic bone marrow failure syndrome. The current treatment options are suboptimal and do not prevent the eventual onset of aplastic anemia requiring bone marrow transplantation. We previously showed that resveratrol, an antioxidant and an activator of the protein deacetylase Sirt1, enhanced hemato...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.05.007
更新日期：2015-07-01 00:00:00
abstract：:Frontotemporal dementia with parkinsonism linked to chromosome 17q21.2 (FTDP-17) is an autosomal-dominant neurodegenerative disorder. Mutations in the MAPT (microtubule-associated protein tau) gene can cause FTDP-17, but the underlying pathomechanisms of the disease are still unknown. Induced pluripotent stem cells (i...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.09.024
更新日期：2016-11-01 00:00:00
abstract：:GABAergic interneuron dysfunction has been implicated in temporal lobe epilepsy (TLE), autism, and schizophrenia. Inhibitory interneuron progenitors transplanted into the hippocampus of rodents with TLE provide varying degrees of seizure suppression. We investigated whether human embryonic stem cell (hESC)-derived int...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.10.007
更新日期：2018-12-01 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：: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：:Human embryonic stem cells (hESCs) offer new avenues for studying human development and disease progression in addition to their tremendous potential toward development of cell-replacement therapies for various cellular disorders. We have earlier reported the derivation and characterization of Relicell(®) hES1, the fi...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.07.001
更新日期：2010-11-01 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：: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：:Human induced pluripotent stem cell (hiPSC) line TCIERi001-A was generated from normal human epidermal keratinocytes (NHEK) primary cell line with the nonintegrating system using Sendai reprogramming kit. Sendai particles were used to deliver the defined transcription factors that included three vector preparations, s...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101590
更新日期：2019-12-01 00:00:00
abstract：:Maple syrup urine disease type Ib (MSUD Ib) is an autosomal recessive genetic metabolic disease caused by homozygous or compound heterozygous mutation in BCKDHB on chromosome 6q14. We generated an induced pluripotent stem cell (iPSC) line from peripheral blood mononuclear cells (PBMCs) of a 5-day-old boy with MSUD Ib ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101579
更新日期：2019-10-01 00:00:00
