MMP-9 and MMP-2 Contribute to Neuronal Cell Death in iPSC Models of Frontotemporal Dementia with MAPT Mutations.
How mutations in the microtubule-associated protein tau (MAPT) gene cause frontotemporal dementia (FTD) remains poorly understood. We generated and characterized multiple induced pluripotent stem cell (iPSC) lines from patients with MAPT IVS10+16 and tau-A152T mutations and a control subject. In cortical neurons differentiated from these and other published iPSC lines,we found that MAPT mutations do not affect neuronal differentiation but increase the 4R/3R tau ratio. Patient neurons had significantly higher levels of MMP-9 and MMP-2 and were more sensitive to stress-induced cell death. Inhibitors of MMP-9/MMP-2 protected patient neurons from stress-induced cell death and recombinant MMP-9/MMP-2 were sufficient to decrease neuronal survival. In tau-A152T neurons,inhibition of the ERK pathway decreased MMP-9 expression. Moreover,ectopic expression of 4R but not 3R tau-A152T in HEK293 cells increased MMP-9 expression and ERK phosphorylation. These findings provide insights into the molecular pathogenesis of FTD and suggest a potential therapeutic target for FTD with MAPT mutations.
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Zhang S et al. (MAR 2017)
Stem cell research 19 31--33
Development of human induced pluripotent stem cell (iPSC) line from a 60year old female patient with multiple schwannoma.
Peripheral blood was collected from a clinically diagnosed 60-year old female patient with multiple schwannoma. Peripheral blood mononuclear cells (PBMCs) were reprogrammed with the Yamanaka KMOS reprogramming factors using the Sendai-virus reprogramming system. The transgene-free iPSC line showed pluripotency verified by immunofluorescent staining for pluripotency markers,and the iPSC line was able to differentiate into the 3 germ layers in vivo. The iPSC line also showed normal karyotype. This in vitro cellular model will be useful for further pathological studies of multiple schwannoma.
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85850
85857
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mTeSR™1
mTeSR™1
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Zhang S et al. (MAR 2017)
Stem cell research 19 43--45
Derivation of human induced pluripotent stem cell (iPSC) line from a 79year old sporadic male Parkinson's disease patient.
Peripheral blood was collected from a clinically diagnosed 79-year old male sporadic Parkinson's disease patient. Peripheral blood mononuclear cells (PBMCs) were reprogrammed with the Yamanaka KMOS reprogramming factors using the Sendai-virus reprogramming system. The transgene-free iPSC line showed pluripotency verified by immunofluorescent staining for pluripotency markers,and the iPSC line was able to differentiate into the 3 germ layers in vivo. The iPSC line also showed normal karyotype. This in vitro cellular model can be used to study the mechanism of sporadic Parkinson's disease and to test new drugs. Resource Table.
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Zhou Y et al. (NOV 2014)
Scientific reports 4 6978
Trend of telomerase activity change during human iPSC self-renewal and differentiation revealed by a quartz crystal microbalance based assay.
Telomerase plays an important role in governing the life span of cells for its capacity to extend telomeres. As high activity of telomerase has been found in stem cells and cancer cells specifically,various methods have been developed for the evaluation of telomerase activity. To overcome the time-consuming procedures and complicated manipulations of existing methods,we developed a novel method named Telomeric Repeat Elongation Assay based on Quartz crystal microbalance (TREAQ) to monitor telomerase activity during the self-renewal and differentiation of human induced pluripotent stem cells (hiPSCs). TREAQ results indicated hiPSCs possess invariable telomerase activity for 11 passages on Matrigel and a steady decline of telomerase activity when differentiated for different periods,which is confirmed with existing golden standard method. The pluripotency of hiPSCs during differentiation could be estimated through monitoring telomerase activity and compared with the expression levels of markers of pluripotency gene via quantitative real time PCR. Regular assessment for factors associated with pluripotency or stemness was expensive and requires excessive sample consuming,thus TREAQ could be a promising alternative technology for routine monitoring of telomerase activity and estimate the pluripotency of stem cells.
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60070
60070.1
60070AD
60070AD.1
60070AZ
60070AZ.1
60070BT.1
60070FI
60070FI.1
60070PE
60070PE.1
60070PS
60093
60093.1
60093AD
60093AD.1
60093PE
60093PE.1
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小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21)
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21)
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),Alexa Fluor® 488
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),Alexa Fluor® 488
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),APC
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),APC
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),Biotin
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),FITC
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),FITC
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),PE
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),PE
小鼠 IgG1,kappa 同型对照抗体(克隆 MOPC-21),PerCP-Cy5.5
抗人OCT4(OCT3)抗体,克隆3A2A20
抗人OCT4(OCT3)抗体,克隆3A2A20
抗人OCT4(OCT3)抗体,克隆3A2A20,Alexa Fluor® 488
抗人OCT4(OCT3)抗体,克隆3A2A20,Alexa Fluor® 488
抗人OCT4(OCT3)抗体,克隆3A2A20,PE
抗人OCT4(OCT3)抗体,克隆3A2A20,PE
mTeSR™1
mTeSR™1
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Galera-Monge T et al. (MAY 2016)
Stem Cell Research 16 3 766--769
Generation of a human iPSC line from a patient with Leigh syndrome caused by a mutation in the MT-ATP6 gene
Human iPSC line L749.1 was generated from fibroblasts of a patient with Leigh syndrome associated with a heteroplasmic mutation in the MT-ATP6 gene. Reprogramming factors OCT4,SOX2,CMYC and KLF4 were delivered using retroviruses.
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85857
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mTeSR™1
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Zhang S et al. (MAR 2017)
Stem cell research 19 49--51
Generation of a human induced pluripotent stem cell (iPSC) line from a 64year old male patient with multiple schwannoma.
Peripheral blood was collected from a clinically diagnosed 64-year old male multiple schwannoma patient. Peripheral blood mononuclear cells (PBMCs) were reprogrammed with the Yamanaka KMOS reprogramming factors using the Sendai-virus reprogramming system. The transgene-free iPSC line showed pluripotency verified by immunofluorescent staining for pluripotency markers,and the iPSC line was able to differentiate into the 3 germ layers in vivo. The iPSC line also showed normal karyotype. This in vitro cellular model will be useful for further pathological studies of multiple schwannoma.
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85850
85857
产品名:
mTeSR™1
mTeSR™1
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Varga E et al. (MAY 2017)
Stem cell research 21 19--22
Establishment of an induced pluripotent stem cell (iPSC) line from a 9-year old male with autism spectrum disorder (ASD).
Peripheral blood mononuclear cells (PBMCs) were collected from a clinically characterized patient with autism spectrum disorder (ASD). The PMBCs were reprogrammed with the human OSKM transcription factors using the Sendai-virus delivery system. The pluripotency of transgene-free iPSCs was verified by immunocytochemistry for pluripotency markers and by spontaneous in vitro differentiation towards the 3 germ layers. Furthermore,the iPSC line showed normal karyotype. Our model might offer a good platform to study the pathomechanism of ASD,also for drug testing,early biomarker discovery and gene therapy studies.
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85857
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mTeSR™1
mTeSR™1
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A. Wilmes et al. ( 2017)
Toxicology in Vitro
Towards optimisation of induced pluripotent cell culture: Extracellular acidification results in growth arrest of iPSC prior to nutrient exhaustion
Human induced pluripotent stem cells (iPSC) have the potential to radically reduce the number of animals used in both toxicological science and disease elucidation. One initial obstacle culturing iPSC is that they require daily medium exchange. This study attempts to clarify why and propose some practical solutions. Two iPSC lineages were fed at different intervals in a full growth area (FGA) or a restricted growth area (RGA). The FGA consisted of a well coated with Matrigel™ and the RGA consisted of a coated coverslip placed in a well. Glucose,lactate,extracellular pH and cell cycle phases were quantified. Without daily feeding,FGA cultured iPSC had significantly reduced growth rates by day 2 and began to die by day 3. In contrast,RGA cultured cells grew to confluence over 3 days. Surprisingly,glucose was not exhausted under any condition. However,extracellular pH reached 6.8 after 72 h in FGA cultures. Artificially reducing medium pH to 6.8 also inhibited glycolysis and initiated an increase in G0/G1 phase of the cell cycle,while adding an additional 10 mM bicarbonate to the medium increased glycolysis rates. This study demonstrates that iPSC are highly sensitive to extracellular acidification,a likely limiting factor in maintenance of proliferative and pluripotent status. Culturing iPSC in RGA prevents rapid extracellular acidification,while still maintaining pluripotency and allowing longer feeding cycles.
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Zhang S et al. (MAR 2017)
Stem cell research 19 34--36
Characterization of human induced pluripotent stem cell (iPSC) line from a 72year old male patient with later onset Alzheimer's disease.
Peripheral blood was collected from a clinically diagnosed 72-year old male patient with later onset Alzheimer's disease. Peripheral blood mononuclear cells (PBMCs) were reprogrammed with the Yamanaka KMOS reprogramming factors using the Sendai-virus reprogramming system. The transgene-free iPSC line showed pluripotency verified by immunofluorescent staining for pluripotency markers,and the iPSC line was able to differentiate into the 3 germ layers in vivo. The iPSC line also showed normal karyotype. This in vitro cellular model will be useful for studying the pathological mechanism of Alzheimer's disease.
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mTeSR™1
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Varga E et al. (OCT 2016)
Stem cell research 17 3 482--484
Generation of Mucopolysaccharidosis type II (MPS II) human induced pluripotent stem cell (iPSC) line from a 1-year-old male with pathogenic IDS mutation.
Peripheral blood was collected from a 1-year-old male patient with an X-linked recessive mutation of Iduronate 2-sulfatase (IDS) gene (NM000202.7(IDS):c.85CtextgreaterT) causing MPS II (OMIM 309900). Peripheral blood mononuclear cells (PBMCs) were reprogrammed by lentiviral delivery of a self-silencing hOKSM polycistronic vector. The pluripotency of the iPSC line was confirmed by the expression of pluripotency-associated markers and in vitro spontaneous differentiation towards the 3 germ layers. The iPSC line showed normal karyotype. The cell line offers a good platform to study MPS II pathophysiology,for drug testing,early biomarker discovery and gene therapy studies.
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Varga E et al. (OCT 2016)
Stem cell research 17 3 514--516
Generation of human induced pluripotent stem cell (iPSC) line from an unaffected female carrier of Mucopolysaccharidosis type II (MPS II) disorder.
Peripheral blood was collected from a 39-year-old unaffected female carrier of an X-linked recessive mutation of Iduronate 2-sulfatase gene (NM000202.7(IDS):c.85CtextgreaterT) causing MPS II (OMIM 309900). Peripheral blood mononuclear cells (PBMCs) were reprogrammed by lentiviral delivery of a self-silencing hOKSM polycistronic vector. The pluripotency of iPSC line was confirmed by the expression of pluripotency-associated markers and in vitro spontaneous differentiation towards the 3 germ layers. The iPSC showed normal karyotype. The line offers a good platform to study MPS II pathophysiology,for drug testing,early biomarker discovery and gene therapy studies.
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85850
85857
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mTeSR™1
mTeSR™1
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Varga E et al. (OCT 2016)
Stem cell research 17 3 531--533
Establishment of EHMT1 mutant induced pluripotent stem cell (iPSC) line from a 11-year-old Kleefstra syndrome (KS) patient with autism and normal intellectual performance.
Peripheral blood was collected from a clinically characterized female Kleefstra syndrome patient with a heterozygous,de novo,premature termination codon (PTC) mutation (NM024757.4(EHMT1):c.3413GtextgreaterA; p.Trp1138Ter). Peripheral blood mononuclear cells (PBMCs) were reprogrammed with the human OSKM transcription factors using the Sendai-virus (SeV) delivery system. The pluripotency of transgene-free iPSC line was verified by the expression of pluripotency-associated markers and by in vitro spontaneous differentiation towards the 3 germ layers. Furthermore,the iPSC line showed normal karyotype. Our model might offer a good platform to study the pathomechanism of Kleefstra syndrome,also for drug testing,early biomarker discovery and gene therapy studies.
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