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 cells of a female patient with a homozygous PLA2G6 c.991G > T (p.D331Y) mutation by using the Sendai-virus delivery system. The resulting iPSCs showed pluripotency confirmed by immunofluorescent staining for pluripotency markers and differentiated into the 3 germ layers in vivo. This cellular model will provide a good resource for further pathophysiological studies of PD.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Cheng YC,Lin HI,Syu SH,Lu HE,Huang CY,Lin CH,Hsieh PCHdoi
10.1016/j.scr.2019.101432subject
Has Abstractpub_date
2019-05-01 00:00:00pages
101432eissn
1873-5061issn
1876-7753pii
S1873-5061(19)30062-5journal_volume
37pub_type
杂志文章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：: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：:Neural stem cells (NSCs) have been considered as potential therapy in Alzheimer's disease (AD) but their use is hampered by the poor survival of grafted cells. Supply of neurotrophic factors to the grafted cells has been proposed as a way to augment survival of the stem cells. In this context, we investigated the util...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.04.008
更新日期：2015-07-01 00:00:00
abstract：:An induced pluripotent stem cell (iPSC) line was generated from a 36-year-old patient with sporadic Parkinson's disease (PD). Skin fibroblasts were reprogrammed using the non-integrating Sendai virus technology to deliver OCT3/4, SOX2, c-MYC and KLF4 factors. The generated cell line (CSC-43) exhibits expression of com...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2018.01.007
更新日期：2018-03-01 00:00:00
abstract：:Expanded human T cells from a Japanese female with recessive dystrophic epidermolysis bullosa (RDBE) were used to generate integration-free induced pluripotent stem cells (iPSCs) by exogenous expression of four reprogramming factors, OCT3/4, SOX2, cMYC, KLF4, using Sendai virus vector (SeVdp). The authenticity of esta...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.05.003
更新日期：2016-07-01 00:00:00
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 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：: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：:Myeloid ecotropic viral integration site 1 (MEIS1) plays an essential role in the development of several embryonic organs, such as the central nervous system and eyes. To further investigate the role of MEIS1 in embryonic development, herein, we generated a MEIS1 homozygous knockout human embryonic stem cell (hESC) li...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102069
更新日期：2020-12-01 00:00:00
abstract：:We have established the patient-specific induced pluripotent stem (iPS) cell line CSUASOi004-A by using peripheral blood mononuclear cells (PBMCs) of a retinitis pigmentosa (RP) patient with a PRPF6 gene mutation (c.G2699A:p.R900H). CSUASOi004-A was established by a non-integrative method with four episomal plasmids c...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101793
更新日期：2020-05-01 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：:Osteogenesis Imperfecta (OI) is a rare autosomal dominant metabolic disorder caused by heterozygous mutations in the COL1A1 or COL1A2 genes, which encode the pro-α1(I) and pro-α2(I) chains of type I procollagen, respectively. A human induced pluripotent stem cell (iPSC) line, termed as CHFUi001-A, was generated from p...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101907
更新日期：2020-07-05 00:00:00
abstract：:Human teratoma is a germ cell tumor that contains normal tissues (e.g., hair, skin or cartilage) differentiated from embryonal germ layers. Because of the feature of this tumor, we hypothesized that human teratomas contain multipotent stem cells that can develop into various non-cancerous normal tissues. In this study...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101633
更新日期：2019-12-01 00:00:00
abstract：:Skin fibroblasts were collected from a 65-year-old male patient with sporadic Parkinson's disease. Induced pluripotent stem cells were reprogrammed with human reprogramming factors (KMOSL) using the messenger RNA reprogramming protocol. The transgene-free iPSC line showed pluripotency, displayed normal karyotype, and ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2017.07.004
更新日期：2017-08-01 00:00:00
abstract：:Recent clinical trials show the efficacy of Adipose-derived Stromal Cells (ASCs) in contrasting the osteoarthritis scenario. Since it is quite accepted that ASCs act predominantly through a paracrine mechanism, their secretome may represent a valid therapeutic substitute. The aim of this study was to investigate the e...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101463
更新日期：2019-07-01 00:00:00
abstract：:Rubinstein-Taybi syndrome (RSTS) is a neurodevelopmental disorder characterized by growth retardation, skeletal anomalies and intellectual disability, caused by heterozygous mutations in either CREBBP (RSTS1) or EP300 (RSTS2) genes. We characterized 3 iPSC lines generated by Sendai from blood of RSTS1 patients with un...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101553
更新日期：2019-10-01 00:00:00
abstract：:Here we utilized the chromatin in vivo assay (CiA) mouse platform to directly examine the epigenetic barriers impeding the activation of the CiA:Oct4 allele in mouse embryonic fibroblasts (MEF)s when stimulated with a transcription factor. The CiA:Oct4 allele contains an engineered EGFP reporter replacing one copy of ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101470
更新日期：2019-07-01 00:00:00
abstract：:The future application of human embryonic stem cells (hESC) for therapeutic approaches requires the development of xeno-free culture conditions to prevent the potential transmission of animal pathogens or xenobiotic substances to hESC. An important component of the majority of hESC culture systems developed is the req...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.08.002
更新日期：2011-01-01 00:00:00
abstract：:The CD71/Ter119 combination has been widely used to reflect dynamic maturation of erythrocytes in vivo. However, because CD71 is expressed on all proliferating cells, it is unclear whether it can be utilized as an erythrocyte-specific marker during differentiation of embryonic stem cells (ESCs). In this study, we reve...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2014.11.002
更新日期：2015-01-01 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：:Cartilage defects have limited capacity for repair and are often replaced by fibrocartilage with inferior mechanical properties. To overcome the limitations of artificial joint replacement, high-throughput screens (HTS) could be developed to identify molecules that stimulate differentiation and/or proliferation of art...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2010.08.007
更新日期：2011-01-01 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：: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：: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：:Human embryonic stem (hES) cells have the dual ability to self-renew and differentiate into specialized cell types. However, in order to realize the full potential of these cells it is important to understand how the genes responsible for their unique characteristics are regulated. In this study we examine the regulat...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2011.10.003
更新日期：2012-03-01 00:00:00
abstract：:GADD45A is a DNA damage and stressful growth arrest inducible protein, also it is shown to a be tumor suppressor gene and a chromatin relaxer associated with opening chromatin during the somatic reprogramming. However, its role in human embryonic stem cells and human embryonic stem cell modeled development has been me...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102090
更新日期：2020-12-01 00:00:00
abstract：:Recently, many hurdles and limitations for production of clinically applicable iPSC derivatives have been overcome. Transgene-free iPSCs can be efficiently derived from easily accessible cell sources such as blood. Here we describe the generation of transgene-free hiPS cells from cord blood derived CD34+ cells, reprog...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2017.03.022
更新日期：2017-05-01 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：: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：: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
