Abstract：
:Among the known causative genes of familial ALS, SOD1mutation is one of the most common. It encodes for the ubiquitous detoxifying copper/zinc binding SOD1 enzyme, whose mutations selectively cause motor neuron death, although the mechanisms are not as yet clear. What is known is that mutant-mediated toxicity is not caused by loss of its detoxifying activity but by a gain-of-function. In order to better understand the pathogenic mechanisms of SOD1 mutation, a human induced pluripotent stem cell (hiPSC) line was generated from the somatic cells of a female patient carrying a missense variation in SOD1 (L145F).
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
D'Anzi A,Altieri F,Perciballi E,Ferrari D,Bernardini L,Goldoni M,Mazzini L,De Marchi F,Di Pierro A,D'Alfonso S,Gelati M,Vescovi AL,Rosati Jdoi
10.1016/j.scr.2020.101924subject
Has Abstractpub_date
2020-07-25 00:00:00pages
101924eissn
1873-5061issn
1876-7753pii
S1873-5061(20)30225-7journal_volume
47pub_type
杂志文章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：: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：: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：: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：:Wolfram Syndrome is a rare, autosomal recessive genetic disorder with clinical symptoms appearing in early childhood. Here, we report a generation of iPSCs from fibroblasts of a patient affected by this disease. Induced pluripotent cells obtained with the application of integration-free episomal vectors display a norm...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101858
更新日期：2020-07-01 00:00:00
abstract：:Primary hepatocyte transplantation (HTx) is a safe cell therapy for patients with liver disease, but wider application is circumvented by poor cell engraftment due to limitations in hepatocyte quality and transplantation strategies. Hepatocyte-like cells (HLCs) derived from human induced pluripotent stem cells (hiPSC)...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101599
更新日期：2019-12-01 00:00:00
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 us...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101621
更新日期：2019-12-01 00:00:00
abstract：:Autism is a heterogeneous neurodevelopmental disorder defined by deficits in socialization, communication, and patterns of behavior. Using stem cells to model brain disordersmay yield new understanding about the underlying neuropathological processes and could prove essential for drug development. We present here a ne...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102038
更新日期：2020-12-01 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：: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：:Asparagine synthetase (ASNS) deficiency (ASNSD; MIM #615574) is a rare neurodevelopmental disorder caused by mutations in the ASNS gene. The ASNS gene maps to cytogenetic band 7q21.3 and is 35 kb long. ASNSD is characterised by congenital microcephaly, severely delayed psychomotor development, seizures, and hyperekple...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101583
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：: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：:We have generated iPSCs from peripheral blood mononuclear cells (PBMCs) of a healthy man using heat sensitive and non-integrative Sendai virus containing Sox2, Oct3/4, c-Myc and Klf4. Human GRX-MCiPS4F-A2 cell line was established and characterized through this study. ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2015.07.002
更新日期：2015-09-01 00:00:00
abstract：:The purpose of this study was to isolate cancer stem cells (CSCs, also called tumor-initiating cells, TICs) from established human colorectal carcinoma (CRC) cell lines, characterize them extensively and dissect the mechanism for their stemness. Freshly isolated CD44(+) and CD44(-) cells from the HCT-15 human colon ca...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2013.09.011
更新日期：2014-01-01 00:00:00
abstract：:The KCL017 human embryonic stem cell line was derived from an embryo donated for research that carried an autosomal dominant mutation affecting splicing site of the VHL gene encoding von Hippel-Lindau tumor suppressor E3 ubiquitin protein ligase (676+3A>T). The ICM was isolated using laser microsurgery and plated on γ...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.01.013
更新日期：2016-03-01 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：: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：:Thrombocytopenia 2 (THC2) is a major type of inherited thrombocytopenia caused by the persistent ANKRD26 expression during the late stage of megakaryocytopoiesis. For the first time, we generated a human induced pluripotent stem cell (hiPSC) line SHAMUi001-A from the bone marrow hematopoietic progenitor cells of a THC...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.102002
更新日期：2020-10-01 00:00:00
abstract：:Stem cells can differentiate into various body tissues and organs and thus are considered as promising tools for cell therapy and tissue engineering. Early passage stem cells have high differentiation ability compared to late passage stem cells. Thus, it is important to use early passage stem cells in cell therapy. He...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2019.101475
更新日期：2019-07-01 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：: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 report here a transgenic murine induced pluripotent stem cell (iPSC) line expressing puromycin N-acetyltransferase (PAC) and enhanced green fluorescent protein (EGFP) under the control of α-myosin heavy chain promoter. This transgenic cell line reproducibly differentiates into EGFP-expressing cardiomyocytes (CMs) w...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2016.07.007
更新日期：2016-09-01 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：:Two iPSC clones, NCCSi008-A and NCCSi008-B, were generated from a healthy male individual of Indian origin by reprogramming his CD4+ T cells with an integration free Sendai viral vector. The established iPSC clones showed high alkaline phosphatase (ALP) activity, expression of pluripotency markers, a normal male karyo...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101765
更新日期：2020-05-01 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 purpose of this study is to establish a murine embryonic stem cell (mESC) line for isolation of functional ventricular cardiomyocytes (VCMs) and then to characterize the derived VCMs. By crossing the myosin light chain 2v (Mlc2v)-Cre mouse line with the reporter strain Rosa26-yellow fluorescent protein (YFP), we g...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2011.08.004
更新日期：2012-01-01 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：:Liver cirrhosis accompanied with hepatic encephalopathy commonly causes cognitive impairment in patients. To model this disease, two independent patient specific induced pluripotent stem cell-line (iPSC) clones, NCCSi011-A and NCCSi011-B were generated by reprogramming the CD4+ T cells of an Indian male patient suffer...
journal_title：Stem cell research
pub_type： 杂志文章
doi：10.1016/j.scr.2020.101992
更新日期：2020-10-01 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
