Integration-free reprogramming of human umbilical arterial endothelial cells into induced pluripotent stem cells IHSTMi001-A
Publication date: Available online 12 August 2018Source: Stem Cell ResearchAuthor(s): Huilin Li, Haiyun Pei, Jiqin Nie, Mingyi Qu, Zeng Fan, Yali Jia, Lijuan He, Xue Nan, Wen Yue, Xuetao PeiAbstractPrimary arterial endothelial cell (AEC) is an attractive source of tissue-engineered blood vessels for therapeutic transplantation in vascular disease. However, scarcity of donor tissue, inability of proliferation and undergo de-differentiation in culture remain major obstacles. We derived a stable induced pluripotent stem cell (iPSC) line possessed all the characteristics of pluripotent state from human umbilical arterial endot...
Source: Stem Cell Research - August 13, 2018 Category: Stem Cells Source Type: research

A naked mole rat iPSC line expressing drug-inducible mouse pluripotency factors developed from embryonic fibroblasts
Publication date: August 2018Source: Stem Cell Research, Volume 31Author(s): Sang-Goo Lee, Aleksei E. Mikhalchenko, Sun Hee Yim, Vadim N. GladyshevAbstractNaked mole rats (NMRs, Heterocephalus glaber) are long-lived, cancer-resistant rodents. Here, we report the development of an induced pluripotent stem cell (iPSC) line generated from immortalized NMR embryonic fibroblasts transduced with a doxycycline-inducible mouse OSKM polycistronic vector. This iPSC line was shown to express pluripotency-associated markers, form embryoid bodies, differentiate in vitro to the derivatives of three germ layers, and exhibit normal karyot...
Source: Stem Cell Research - August 12, 2018 Category: Stem Cells Source Type: research

Three lines of induced pluripotent stem cells derived from a 15q11.2-q13.1 duplication syndrome patient
Publication date: Available online 10 August 2018Source: Stem Cell ResearchAuthor(s): Yuko Arioka, Itaru Kushima, Daisuke Mori, Norio OzakiAbstractDuplications at the 15q11.2-q13.1 region are associated with psychiatric disorders such as developmental delay and autism spectrum disorder. However, the specific influence of these duplications on human neuronal cells remains unclear. Here we generated induced pluripotent stem cells (iPSCs) derived from a patient with 15q11.2-q13.1 duplication syndrome. The generated iPSCs carried 15q11.2-q13.1 duplication and showed typical iPSC morphology and pluripotency marker expression, a...
Source: Stem Cell Research - August 11, 2018 Category: Stem Cells Source Type: research

Generation of the human induced pluripotent stem cell line (UKWNLi001-A) from skin fibroblasts of a woman with Fabry disease carrying the X-chromosomal heterozygous c.708 G > C (W236C) missense mutation in exon 5 of the alpha-galactosidase–A gene
Publication date: Available online 10 August 2018Source: Stem Cell ResearchAuthor(s): Thomas Klein, Katharina Günther, Chee Keong Kwok, Frank Edenhofer, Nurcan ÜçeylerAbstractHuman dermal fibroblasts (HDF) were isolated from the skin punch biopsy of a 25-year-old woman with Fabry disease (FD), carrying a heterozygous c.708 G > C missense mutation in the alpha-galactosidase A gene. HDF were reprogrammed to induced pluripotent stem cells (iPSC) using synthetic mRNA, preventing the alteration of the genome and retaining the original genotype. FD-W236C-iPSC (UKWNLi001-A) showed typical human embryonic...
Source: Stem Cell Research - August 11, 2018 Category: Stem Cells Source Type: research

Generation of induced pluripotent stem cells, KCi001-A derived from a Bardet-Biedl syndrome patient compound heterozygous for the BBS1 variants c.1169T>G/c.1135G>C
Publication date: Available online 10 August 2018Source: Stem Cell ResearchAuthor(s): Caroline Amalie Brunbjerg Hey, Katarina Beata Saltõkowa, Lasse Jonsgaard Larsen, Zeynep Tümer, Karen Brøndum-Nielsen, Karen Grønskov, Tina Duelund Hjortshøj, Lisbeth Birk MøllerAbstractBardet-Biedl syndrome (BBS) is an autosomal recessive ciliopathy with a wide range of symptoms including obesity, retinal dystrophy, polycystic kidney disease, polydactyly, hypogonadism and learning difficulties. Here we describe the successful generation of an induced pluripotent stem cell (iPSC) KCi001-A from a BBS ...
Source: Stem Cell Research - August 11, 2018 Category: Stem Cells Source Type: research

Odd skipped-related 1 (Osr1) identifies muscle-interstitial fibro-adipogenic progenitors (FAPs) activated by acute injury
In conclusion, reporter LacZ or eGFP-CreERt2 expression from the endogenous Osr1 locus serves as marker for FACS isolation and tamoxifen-induced manipulation of activated FAPs. (Source: Stem Cell Research)
Source: Stem Cell Research - August 10, 2018 Category: Stem Cells Source Type: research

Improving single-cell cloning workflow for gene editing in human pluripotent stem cells
Publication date: Available online 4 August 2018Source: Stem Cell ResearchAuthor(s): Yi-Hsien Chen, Shondra M. Pruett-MillerAbstractThe availability of human pluripotent stem cells (hPSCs) and progress in genome engineering technology have altered the way we approach scientific research and drug development screens. Unfortunately, the procedures for genome editing of hPSCs often subject cells to harsh conditions that compromise viability: a major problem that is compounded by the innate challenge of single-cell culture. Here we describe a generally applicable workflow that supports single-cell cloning and expansion of hPSC...
Source: Stem Cell Research - August 4, 2018 Category: Stem Cells Source Type: research

Establishment of an induced pluripotent stem cell line from a retinitis pigmentosa patient with compound heterozygous CRB1 mutation
Publication date: Available online 2 August 2018Source: Stem Cell ResearchAuthor(s): Xiao Zhang, Dan Zhang, Shang-Chih Chen, Tina Lamey, Jennifer A. Thompson, Terri McLaren, John N. De Roach, Fred K. Chen, Samuel McLenachanAbstractThe 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. LEIi006-A expressed pluripotent stem cell markers, had a normal karyo...
Source: Stem Cell Research - August 3, 2018 Category: Stem Cells Source Type: research

Establishment of a human iPSC line, IISHDOi004-A, from a patient with Usher syndrome associated with the mutation c.2276G>T; p.Cys759Phe
Publication date: Available online 2 August 2018Source: Stem Cell ResearchAuthor(s): Francisco Zurita-Díaz, María del Carmen Ortuño-Costela, Ana Moreno-Izquierdo, Liliana Galbis, José María Millán, Carmen Ayuso, Rafael Garesse, M. Esther GallardoAbstractA human iPSC line, IISHDOi004-A, from fibroblasts obtained from a patient with Usher syndrome, harboring a homozygous mutation in the USH2A gene (c.2276G>T; p.Cys759Phe) has been generated. Reprogramming factors Oct3/4, Sox2, Klf4, and c-Myc were delivered using Sendai virus. (Source: Stem Cell Research)
Source: Stem Cell Research - August 3, 2018 Category: Stem Cells Source Type: research

Generation of a human induced pluripotent stem cell line (LL008 1.4) from a familial Alzheimer's disease patient carrying a double KM670/671NL (Swedish) mutation in APP gene
Publication date: Available online 1 August 2018Source: Stem Cell ResearchAuthor(s): Minna Oksanen, Ida Hyötyläinen, Jenni Voutilainen, Katja A. Puttonen, Riikka H. Hämäläinen, Caroline Graff, Šárka Lehtonen, Jari KoistinahoAbstractA double mutation (KM670/671NL) in amyloid precursor protein gene (APP) is causative for familial Alzheimer's disease and has been shown to increase the total Aβ burden. Here we report the generation and characterization of an iPSC line from a fAD patient carrying APP KM670/671NL. The generated iPSCs retained the mutation, expressed pluripotency marker...
Source: Stem Cell Research - August 2, 2018 Category: Stem Cells Source Type: research

Meticulous optimization of cardiomyocyte yields in a 3-stage continuous integrated agitation bioprocess
Publication date: Available online 1 August 2018Source: Stem Cell ResearchAuthor(s): Sherwin Ting, Alan Lam, Gerine Tong, Allen Chen, Heming Wei, JianJun Wu, Yue Ning Lam, Shaul Reuveny, Steve OhAbstractHuman pluripotent stem cells (hPSCs) can be a renewable source for generating cardiomyocyte (CM) for treating myocardial infraction. In our previous publication, we described an integrated microcarrier-based wave reactor process for the expansion and differentiation of hPSCs to CMs on a rocker based platform. However, this platform is limited in terms of linear scalability and CMs purity. The present study describes ways to...
Source: Stem Cell Research - August 2, 2018 Category: Stem Cells Source Type: research

NOSTRIN: A novel modulator of trophoblast giant cell differentiation
Publication date: Available online 29 July 2018Source: Stem Cell ResearchAuthor(s): Shreeta Chakraborty, Rupasri AinAbstractDifferentiation-dependent expression of NOSTRIN in murine trophoblast cells prompted investigation on NOSTRIN's function in trophoblast differentiation. We show here that NOSTRIN levels increased in both mouse and rat placenta during gestation. NOSTRIN expression was not co-related to expression of eNOS precluding its eNOS mediated function. NOSTRIN transcripts were identified in trophoblast cells of the placenta, predominantly in trophoblast giant cells (TGC). Precocious over-expression of NOSTRIN du...
Source: Stem Cell Research - July 30, 2018 Category: Stem Cells Source Type: research

A single cell transcriptional portrait of embryoid body differentiation and comparison to progenitors of the developing embryo
Publication date: Available online 29 July 2018Source: Stem Cell ResearchAuthor(s): Abby Spangler, Emily Su, April M. Craft, Patrick CahanAbstractDirected differentiation of pluripotent stem cells provides an accessible system to model development. However, the distinct cell types that emerge, their dynamics, and their relationship to progenitors in the early embryo has been difficult to characterize because of the cellular heterogeneity inherent to differentiation. Here, we used a combination of bulk RNA-Seq, single cell RNA-Seq, and bioinformatics analyses to dissect the cell types that emerge during directed differentia...
Source: Stem Cell Research - July 30, 2018 Category: Stem Cells Source Type: research

Generation of nine induced pluripotent stem cell lines as an ethnic diversity panel
Publication date: Available online 27 July 2018Source: Stem Cell ResearchAuthor(s): Xiugong Gao, Jeffrey J. Yourick, Robert L. SprandoAbstractHuman induced pluripotent stem cells (iPSCs) provide a potentially unlimited source of differentiated cells from individuals with specific genetic backgrounds. Using self-replicative RNA reprogramming technology, we generated nine iPSC lines from endothelial progenitor cells (EPCs) derived from blood samples of three different ethnicities: Black or African American, Latino or Hispanic, and Non-Hispanic White. The resulting iPSC lines showed normal karyotype in large part, expressed p...
Source: Stem Cell Research - July 28, 2018 Category: Stem Cells Source Type: research

Generation of a human iPSC line from a patient with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) caused by mutation in SACSIN gene
Publication date: Available online 27 July 2018Source: Stem Cell ResearchAuthor(s): Candela Machuca Arellano, Angel Vilches, Eleonora Clemente, Samuel Ignacio Pascual-Pascual, Arantxa Bolinches-Amorós, Ana Artero Castro, Carmen Espinos, Marian Leon Rodriguez, Pavla Jendelova, Slaven ErcegAbstractThe human iPSC cell line, ARS-FiPS4F1 (ESi063-A), derived from dermal fibroblast from the patient autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) caused by mutations on the gene SACSIN, was generated by non-integrative reprogramming technology using OCT3/4, SOX2, CMYC and KLF4 reprogramming factors. The pl...
Source: Stem Cell Research - July 27, 2018 Category: Stem Cells Source Type: research

Establishment and characterization of an iPSC line from a 58 years old high grade patient with nonalcoholic fatty liver disease (70% steatosis) with homozygous wildtype PNPLA3 genotype
Publication date: Available online 27 July 2018Source: Stem Cell ResearchAuthor(s): Nina Graffmann, Martina Bohndorf, Audrey Ncube, Wasco Wruck, Karl Kashofer, Kurt Zatloukal, James AdjayeAbstractNonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome and its prevalence increases continuously. Here, we reprogrammed fibroblasts of a high grade NAFLD patient with homozygous wildtype PNPLA3 genotype. We characterized the induced pluripotent stem cells (iPSCs) by immunocytochemistry, flow cytometry, embryoid body formation, pluritest DNA-fingerprinting, and karyotype analysis. (Source: Stem Cell Research)
Source: Stem Cell Research - July 27, 2018 Category: Stem Cells Source Type: research

Derivation of induced pluripotent stem cells TUSMi006 from an 87-year old Chinese Han Alzheimer's disease patient carrying GRINB and SORL1 mutations
Publication date: Available online 25 July 2018Source: Stem Cell ResearchAuthor(s): Ying Wang, Hongxiang Yu, Ying Chen, Gang Li, Ying Lei, Jian ZhaoAbstractA 87-year old Alzheimer's Disease(AD) male patient donated his Peripheral blood mononuclear cells (PBMC). The non-integrating episomal vector system was used to reprogram PBMCs with the human OKSM transcription factors. The pluripotency of transgene-free iPSCs was confirmed by immunocytochemistry for pluripotency markers and by the ability of the iPSCs to differentiate spontaneously into 3 germ layers in vitro. In addition, the iPSC line displayed a normal karyotype. Ou...
Source: Stem Cell Research - July 26, 2018 Category: Stem Cells Source Type: research

Induced pluripotent stem cell line from an atopic dermatitis patient heterozygous for c.2282del4 mutation in filaggrin: KCLi001-A
Publication date: Available online 25 July 2018Source: Stem Cell ResearchAuthor(s): Liani Devito, Matthew Donne, Nikola Kolundzic, Preeti Khurana, Carl Hobbs, Gabriel Kaddour, Sandrine Dubrac, Robert Gruber, Matthias Schmuth, Thea Mauro, Dusko IlicAbstractWe have generated an induced pluripotent stem cell (iPSC) line KCLi001-A (iOP118) from a female atopic dermatitis (AD) patient, heterozygous for the loss-of-function mutation c.2282del4 in the filaggrin gene (FLG). Epidermal keratinocytes were reprogrammed using non-integrating Sendai virus vectors. The entire process of derivation and expansion of AD-iPSCs were performed...
Source: Stem Cell Research - July 26, 2018 Category: Stem Cells Source Type: research

Trehalose to cryopreserve human pluripotent stem cells
This study provides evidence that pluripotent and neural stem cells stored in trehalose alone or with other cryoprotectants (CPAs) maintain their functional properties, indicating their potential use in cell therapies if produced in good manufacturing practice (GMP) facility. (Source: Stem Cell Research)
Source: Stem Cell Research - July 25, 2018 Category: Stem Cells Source Type: research

Establishment and characterization of an iPSC line from a 35 years old high grade patient with nonalcoholic fatty liver disease (30–40% steatosis) with homozygous wildtype PNPLA3 genotype
Publication date: Available online 25 July 2018Source: Stem Cell ResearchAuthor(s): Nina Graffmann, Martina Bohndorf, Audrey Ncube, Marie-Ann Kawala, Wasco Wruck, Karl Kashofer, Kurt Zatloukal, James AdjayeAbstractNonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome and its prevalence increases continuously. Here, we reprogrammed fibroblasts of a high grade NAFLD patient with homozygous wildtype PNPLA3 genotype. The induced pluripotent stem cells (iPSCs) were characterized by immunocytochemistry, flow cytometry, embryoid body formation, pluritest, DNA-fingerprinting and karyotype ...
Source: Stem Cell Research - July 25, 2018 Category: Stem Cells Source Type: research

Generation of patient-specific induced pluripotent stem cell lines from one patient with Jervell and Lange-Nielsen syndrome, one with type 1 long QT syndrome and two healthy relatives
Publication date: Available online 25 July 2018Source: Stem Cell ResearchAuthor(s): T.H. Kasai-Brunswick, D. Silva dos Santos, R.P. Ferreira, D.S. Araujo, G.M. Dias, J.L.A. Coutinho, F.E.S.F. Cruz, E.B. Sternick, F. Gubert, J.C.G. Oliveira, I.M. Vaz, T. Borgonovo, P.R.S. Brofman, R.S. Moura-Neto, R. Silva, A.C. Campos-de-Carvalho, A.B. CarvalhoAbstractFour human iPSC cell lines (one Jervell and Lange-Nielsen Syndrome, one Long QT Syndrome-type 1 and two healthy controls) were generated from peripheral blood obtained from donors belonging to the same family. CytoTune™-iPS 2.0 Sendai Reprogramming Kit (containing OCT3/...
Source: Stem Cell Research - July 25, 2018 Category: Stem Cells Source Type: research

Generation of cytochrome P450 polymorphic human induced pluripotent stem cell lines with defective CYP activities
Publication date: Available online 25 July 2018Source: Stem Cell ResearchAuthor(s): Jaehun Lee, Dong-Hun Woo, Han-Jin Park, Duck Sung Ko, Jong-Hoon KimAbstractSingle nucleotide polymorphisms (SNPs) in cytochrome P450 (CYP) isoenzymes alter drug metabolism and pharmacodynamics. In particular, several SNPs within CYPs decrease CYP activities, resulting in a high plasma concentration of drugs and increasing adverse effect of commonly used drugs. Here, we generated two different human induced pluripotent stem cell (hiPSC) lines, which retain defective CYP2C19 or CYP3A5 activities individually. These two hiPSC lines could be va...
Source: Stem Cell Research - July 25, 2018 Category: Stem Cells Source Type: research

Skin-derived precursor cells undergo substrate-dependent galvanotaxis that can be modified by neighbouring cells
Publication date: Available online 19 July 2018Source: Stem Cell ResearchAuthor(s): Stephanie N. Iwasa, Milos R. Popovic, Cindi M. MorsheadAbstractMany cell types respond to electric fields (EFs) through cell migration, a process termed galvanotaxis. The galvanotactic response is critical for development and wound healing. Here we investigated whether skin-derived precursor cells (SKPs), which have the potential to differentiate into mesodermal and peripheral neural cell types, undergo directed migration in the presence of an EF. We found that EF application promotes SKP migration towards the anode. The migratory response ...
Source: Stem Cell Research - July 20, 2018 Category: Stem Cells Source Type: research

Genomic functions of developmental pluripotency associated factor 4 (Dppa4) in pluripotent stem cells and cancer
In this study, we use ChIP-Seq to identify Dppa4 binding genome-wide in three distinct cell types: mouse embryonic stem cells (mESC), embryonal carcinoma cells, and 3T3 fibroblasts ectopically expressing Dppa4. We find a core set of Dppa4 binding sites shared across cell types, and also a substantial number of sites unique to each cell type. Across cell types Dppa4 shows a preference for binding to regions with active chromatin signatures, and can influence chromatin modifications at target genes. In 3T3 fibroblasts with enforced Dppa4 expression, Dppa4 represses the cell cycle inhibitor Cdkn2c and activates Ets family tra...
Source: Stem Cell Research - July 20, 2018 Category: Stem Cells Source Type: research

Induced pluripotent stem cell line MUSIi006-A derived from hair follicle keratinocytes as a non-invasive somatic cell source
In this study, we used hair follicle keratinocytes for reprogramming. Collection of plucked hairs offers advantages over other somatic cells because no medical professional or operation room is required. Keratinocytes were isolated from plucked hairs of a 21-year-old healthy woman and characterized for the expression of cytokeratin 14 (CK14). Reprogramming of keratinocytes was performed using Sendai virus. Further characterization of the keratinocyte-derived iPSC line (designated as MUSIi006-A) confirmed that the cell line was pluripotent, free from Sendai viral genome and transgenes, and retained normal karyotype. Our met...
Source: Stem Cell Research - July 20, 2018 Category: Stem Cells Source Type: research

Significant therapeutic effects of adult human multipotent neural cells on spinal cord injury
In this study, we investigated the therapeutic efficacy and treatment mechanism of ahMNCs for SCI using rodent models. When 1 × 106 ahMNCs were transplanted into injured spinal cords at 7 days after contusion, the injection group showed significantly better functional recovery than the control group (media injection after contusion), which was determined by the Basso, Beattie and Bresnahan (BBB) score. Although transplanted ahMNCs disappeared continuously, remained cells expressed differentiated neural cell markers (Tuj1) or astrocyte marker (GFAP) in the injured spinal cords. Moreover, the number of CD31-posit...
Source: Stem Cell Research - July 20, 2018 Category: Stem Cells Source Type: research

Differentiation of human iPSCs into VSMCs and generation of VSMC-derived calcifying vascular cells
Publication date: August 2018Source: Stem Cell Research, Volume 31Author(s): Anja Trillhaase, Undine Haferkamp, Alexandra Rangnau, Marlon Märtens, Beatrice Schmidt, Michaela Trilck, Philip Seibler, Redouane Aherrahrou, Jeanette Erdmann, Zouhair AherrahrouAbstractVascular calcification displays a major cause of death worldwide, which involve mainly vascular smooth muscle cells (VSMCs). Since 2007, there are increasing numbers of protocols to obtain different cell types from human induced-pluripotent stem cells (iPSCs), however a protocol for calcification is missing.Few protocols exist today for the differentiation of ...
Source: Stem Cell Research - July 18, 2018 Category: Stem Cells Source Type: research

Generation of ZZUi008-A, a transgene-free, induced pluripotent stem cell line derived from chorionic villi cells of a fetus with Duchenne muscular dystrophy
Publication date: Available online 17 July 2018Source: Stem Cell ResearchAuthor(s): Xiaofan Zhu, Aojie Cai, Jingjing Meng, Lina Liu, Siying Cui, Suzhen Qu, Junhong Zhao, Ning Liu, Xiangdong KongAbstractDuchenne muscular dystrophy (DMD) is a common X-linked recessive disorder for which there is no present cure. In this paper, we reported the generation of ZZUi008-A, an induced pluripotent stem cell(iPSC) line derived from chorionic villus(CV) cells of a fetus with a deletion mutation in exon 33 of the dystrophin gene (DMD). The cell line was generated using feeder-free and virus-free conditions, and the established cell lin...
Source: Stem Cell Research - July 18, 2018 Category: Stem Cells Source Type: research

Editorial Board
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): (Source: Stem Cell Research)
Source: Stem Cell Research - July 14, 2018 Category: Stem Cells Source Type: research

Corrigendum to Generation of a human iPSC line by mRNA reprogramming. Stem Cell Res., Volume 28, April 2018, Pages 157–160
Publication date: August 2018Source: Stem Cell Research, Volume 31Author(s): Ana Artero Castro, Marian León, Noelia Luna-Peláez, Angeles Martín Bernal, Verónica del Buey Furió, Slaven Erceg, Dunja Lukovic (Source: Stem Cell Research)
Source: Stem Cell Research - July 14, 2018 Category: Stem Cells Source Type: research

Distinct requirements for the maintenance and establishment of mouse embryonic stem cells
In conclusion, 2iL-dependent activation of these X-linked genes at the earliest phase of ESC derivation is one of the molecular bases for the acquisition of permanent pluripotency. (Source: Stem Cell Research)
Source: Stem Cell Research - July 12, 2018 Category: Stem Cells Source Type: research

Generation of integration-free induced pluripotent stem cells from a patient with spina bifida
Publication date: Available online 17 May 2018Source: Stem Cell ResearchAuthor(s): Hongran Wang, Shuying Zhao, Richard H. Finnell, Timothy George, Austin J. CooneyAbstractA 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 integrated reprogramming genes, had a stable normal karyotype and expressed pluripotency markers. The iPSCs formed teratomas in mice, which were di...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

A naked mole-rat iPSC line expressing drug-inducible mouse pluripotency factors developed from embryonic fibroblasts
Publication date: Available online 20 June 2018Source: Stem Cell ResearchAuthor(s): Sang-Goo Lee, Aleksei E. Mikhalchenko, Sun Hee Yim, Vadim N. GladyshevAbstractNaked mole rats (NMRs, Heterocephalus glaber) are long-lived, cancer-resistant rodents. Here, we report the development of an induced pluripotent stem cell (iPSC) line generated from immortalized NMR embryonic fibroblasts transduced with a doxycycline-inducible mouse OSKM polycistronic vector. This iPSC line was shown to express pluripotency-associated markers, form embryoid bodies, differentiate in vitro to the derivatives of three germ layers, and exhibit normal...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Generation of the Rubinstein-Taybi syndrome type 2 patient-derived induced pluripotent stem cell line (IAIi001-A) carrying the EP300 exon 23 stop mutation c.3829A > T, p.(Lys1277*)
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Valentina Alari, Silvia Russo, Davide Rovina, Aoife Gowran, Maria Garzo, Milena Crippa, Laura Mazzanti, Claudia Scalera, Ennio Prosperi, Daniela Giardino, Cristina Gervasini, Palma Finelli, Giulio Pompilio, Lidia LarizzaAbstractRubinstein-Taybi syndrome (RSTS) is a neurodevelopmental disorder characterized by growth retardation, skeletal anomalies and intellectual disability, caused by heterozygous mutation in either the CREBBP (RSTS1) or EP300 (RSTS2) genes. We generated an induced pluripotent stem cell line from an RSTS2 patient's blood mononuclea...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Generation of an induced pluripotent stem cell line from a patient with adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP): HIHCNi003-A
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Stefanie Nicole Hayer, Yvonne Schelling, Philip Hoeflinger, Stefan Hauser, Ludger SchölsAbstractAn induced pluripotent stem cell line, HIHCNi003-A (iPSC-ALSP), was created from a skin biopsy of a patient with adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) caused by a heterozygous c.2512G>C, p.Val838Leu mutation in the CSF1R gene. Skin fibroblasts were reprogrammed using episomal plasmids carrying hOCT4, hSOX2, hKLF4, hL-MYC, and hLIN28. The iPSC-ALSP line exhibits chromosomal stability with conservation of th...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Developing two reference control samples for the Indian population
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Shruti Iyer, Priyanka Bhatia, Mahendra Rao, Odity MukherjeeAbstractHuman induced Pluripotent Stem Cells (HiPSCs) have immense potential in research and therapeutics. Under the aegis of Department of Biotechnology funded national program entitled, “The Accelerator program for Discovery in Brain Disorders using Stem Cells (ADBS)” we have established a HiPSC biorepository (https://www.ncbs.res.in/adbs/bio-repository) with an objective to study severe mental illness. The repository comprises of HiPSC lines derived from healthy control donors...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Generation of two isogenic human induced pluripotent stem cell lines from a 15 year-old female patient with MERRF syndrome and A8344G mutation of mitochondrial DNA
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Shih-Jie Chou, Yu-Ling Ko, Yu-Hsuan Yang, Aliaksandr A. Yarmishyn, Yu-Ting Wu, Chien-Tsun Chen, Hsin-Chen Lee, Yau-Huei Wei, Shih-Hwa ChiouAbstractMERRF syndrome is predominantly caused by A8344G mutation in the mitochondrial DNA (mtDNA), affecting MT-TK gene, which impairs the mitochondrial electron transport chain function. Here, we report the generation of two isogenic induced pluripotent stem cell (iPSC) lines, TVGH-iPSC-MRF-Mlow and TVGH-iPSC-MRF-Mhigh, from the skin fibroblasts of a female MERRF patient harboring mtDNA A8344G mutation by using...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Chondrogenic differentiation in vitro of hiPSCs activates pathways engaged in limb development
In this study, we compared the gene expression profile of chondrocyte-like cells differentiated from hiPSCs via monolayer culture (ChiPS) to the profile of mature chondrocytes and to a line of hiPSCs created by our group (GPCCi001-A). Our results indicate that ChiPS possess features of early chondrocytes. This finding was confirmed by RT-qPCR analysis, which demonstrated that the ALX1, EYA1, HOXB6, HOXC11, HOXD13 and RARB genes were more highly expressed in the ChiPS versus both GPCCi001-A cells and adult chondrocytes. These findings provide a better understanding the processes directing the cell fate of hiPSCs during chon...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Efficient and high yield isolation of myoblasts from skeletal muscle
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Aref Shahini, Kalyan Vydiam, Debanik Choudhury, Nika Rajabian, Thy Nguyen, Pedro Lei, Stelios T. AndreadisAbstractSkeletal muscle (SkM) regeneration relies on the activity of myogenic progenitors that reside beneath the basal lamina of myofibers. Here, we describe a protocol for the isolation of the SkM progenitors from young and old mice by exploiting their outgrowth potential from SkM explants on matrigel coated dishes in the presence of high serum, chicken embryo extract and basic fibroblast growth factor. Compared to other protocols, this method...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

NEK1 loss-of-function mutation induces DNA damage accumulation in ALS patient-derived motoneurons
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Julia Higelin, Alberto Catanese, Lena Luisa Semelink-Sedlacek, Sertap Oeztuerk, Anne-Kathrin Lutz, Julia Bausinger, Gotthold Barbi, Günter Speit, Peter M. Andersen, Albert C. Ludolph, Maria Demestre, Tobias M. BoeckersAbstractMutations in genes coding for proteins involved in DNA damage response (DDR) and repair, such as C9orf72 and FUS (Fused in Sarcoma), are associated with neurodegenerative diseases and lead to amyotrophic lateral sclerosis (ALS). Heterozygous loss-of-function mutations in NEK1 (NIMA-related kinase 1) have also been recently...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Endothelial cells instruct liver specification of embryonic stem cell-derived endoderm through endothelial VEGFR2 signaling and endoderm epigenetic modifications
This study provides an efficient 2D platform modelling the endothelial cell niche crosstalk with endoderm, and reveals mechanisms by which endothelial cells promote hepatic specification of mouse ESC-derived endoderm cells through endothelial VEGFR2 activation and endoderm epigenetic modifications. (Source: Stem Cell Research)
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Computer simulation of neutral drift among limbal epithelial stem cells of mosaic mice
In this study we used computer simulations to show that these results could also be explained by stochastic replacement of LESCs by neighbouring LESCs, leading to neutral drift of LESC populations. This was shown to reduce the number of coherent clones of LESCs and hence would coarsen the mosaic pattern in the corneal epithelium without reducing the absolute number of LESCs. Simulations also showed that corrected stripe numbers declined more slowly when LESCs were grouped non-randomly and that mosaicism was rarely lost unless simulated LESC numbers were unrealistically low. Possible reasons why age-related changes differ b...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Derivation and characterization of a UCP1 reporter human ES cell line
This reporter cell line thus presents new opportunities to study human brown fat biology by enabling future work to understand early human brown fat development, perform disease modeling, and facilitate drug screening. (Source: Stem Cell Research)
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Metabolic plasticity during transition to naïve-like pluripotency in canine embryo-derived stem cells
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): I.C. Tobias, R.R. Isaac, J.G. Dierolf, R. Khazaee, R.C. Cumming, D.H. BettsAbstractPluripotent stem cells (PSCs) have been described in naïve or primed pluripotent states. Domestic dogs are useful translational models in regenerative medicine, but their embryonic stem cells (cESCs) remain narrowly investigated. Primed-like cESCs expanded in the presence of leukemia inhibitory factor and fibroblast growth factor 2 (LIF-FGF2) acquire features of naïve pluripotency when exposed to chemical inhibitors and LIF (2iL). However, proliferation of c...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

CD133+ cells derived from skeletal muscles of Duchenne muscular dystrophy patients have a compromised myogenic and muscle regenerative capability
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Jinhong Meng, Francesco Muntoni, Jennifer MorganAbstractCell-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 missing dystrophin protein, thus improving muscle function.CD133+ cells derived from normal human skeletal muscle contribute to regenerated muscle fibres and form muscle stem cell...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Motor neuron differentiation of iPSCs obtained from peripheral blood of a mutant TARDBP ALS patient
In conclusion, we here demonstrated for the first time that human TARDBP mutated MNs can be successfully obtained exploiting the reprogramming and differentiation ability of peripheral blood cells, an easily accessible source from any patient. (Source: Stem Cell Research)
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Induction of quiescence (G0) in bone marrow stromal stem cells enhances their stem cell characteristics
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Mohammad Rumman, Abhijit Majumder, Linda Harkness, Balu Venugopal, M.B. Vinay, Malini S. Pillai, Moustapha Kassem, Jyotsna DhawanAbstractSeveral studies have suggested that bone marrow stromal steam cells (BMSC) exist in a quiescent state (G0) within the in vivo niche; however, an explicit analysis of the biology of G0 state-BMSC has not been reported. We hypothesized that induction of G0 in BMSC might enhance their stem cell properties. Thus, we induced quiescence in BMSC in vitro by (a) suspension culture in a viscous medium or (b) culture on soft...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Inverse agonism of retinoic acid receptors directs epiblast cells into the paraxial mesoderm lineage
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Ryan P. Russell, Yu Fu, Yaling Liu, Peter MayeAbstractWe have investigated the differentiation of paraxial mesoderm from mouse embryonic stem cells utilizing a Tbx6-EYFP/Brachyury (T)-Cherry dual reporter system. Differentiation from the mouse ESC state directly into mesoderm via Wnt pathway activation was low, but augmented by treatment with AGN193109, a pan-retinoic acid receptor inverse agonist. After five days of differentiation, T+ cells increased from 12.2% to 18.8%, Tbx6+ cells increased from 5.8% to 12.7%, and T+/Tbx6+ cells increased from 2...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

Isolation of primitive mouse extraembryonic endoderm (pXEN) stem cell lines
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Yixiang Zhong, Taewoong Choi, Minjae Kim, Kyoung Hwa Jung, Young Gyu Chai, Bert BinasAbstractMouse blastocysts contain the committed precursors of the extraembryonic endoderm (ExEn), which express the key transcription factor Oct4, depend on LIF/LIF-like factor-driven Jak/Stat signaling, and initially exhibit lineage plasticity. Previously described rat blastocyst-derived ExEn precursor-like cell lines (XENP cells/HypoSCs) also show these features, but equivalent mouse blastocyst-derived cell lines are lacking. We now present mouse blastocyst-derive...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research

iPSC-derived neurons of CREBBP- and EP300-mutated Rubinstein-Taybi syndrome patients show morphological alterations and hypoexcitability
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Valentina Alari, Silvia Russo, Benedetta Terragni, Paola Francesca Ajmone, Alessandra Sironi, Ilaria Catusi, Luciano Calzari, Daniela Concolino, Rosa Marotta, Donatella Milani, Daniela Giardino, Massimo Mantegazza, Cristina Gervasini, Palma Finelli, Lidia LarizzaAbstractRubinstein-Taybi syndrome (RSTS) is a rare neurodevelopmental disorder characterized by distinctive facial features, growth retardation, broad thumbs and toes and mild to severe intellectual disability, caused by heterozygous mutations in either CREBBP or EP300 genes, encoding the ho...
Source: Stem Cell Research - July 10, 2018 Category: Stem Cells Source Type: research