1,418 results match your criteria Stem Cell Research [Journal]


Generation of an induced pluripotent stem cell line (TRNDi004-I) from a Niemann-Pick disease type B patient carrying a heterozygous mutation of p.L43_A44delLA in the SMPD1 gene.

Stem Cell Res 2019 Apr 12;37:101436. Epub 2019 Apr 12.

National Center for Advancing Translational Sciences, National Institutes of Health, Bethesda, MD, USA. Electronic address:

Niemann-Pick disease type B (NPB) is a rare autosomal recessive lysosomal storage disease caused by mutations in the SMPD1 gene, which encodes for acid sphingomyelinase. A human induced pluripotent stem cell (iPSC) line was generated from dermal fibroblasts of a 1-year old male patient with NPB that has a heterozygous mutation of a p.L43_A44delLA of SMPD1 using non-integrating Sendai virus technique. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101436DOI Listing

Generation of the human induced pluripotent stem cell (hiPSC) line PSMi005-A from a patient carrying the KCNQ1-R190W mutation.

Stem Cell Res 2019 Apr 13;37:101437. Epub 2019 Apr 13.

Coronary Care Unit, Laboratory of Experimental Cardiology for Cell and Molecular Therapy, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; Department of Molecular Medicine, Unit of Cardiology, Università degli studi di Pavia, Pavia, Italy; Department of Medicine, University of Cape Town, Cape Town, South Africa. Electronic address:

We generated human induced pluripotent stem cells (hiPSCs) from dermal fibroblasts of a woman carrier of the heterozygous mutation c.568C > T p.R190W on the KCNQ1 gene. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S18735061193006
Publisher Site
http://dx.doi.org/10.1016/j.scr.2019.101437DOI Listing
April 2019
2 Reads

Cyclic tensile stress promotes osteogenic differentiation of adipose stem cells via ERK and p38 pathways.

Stem Cell Res 2019 Apr 8;37:101433. Epub 2019 Apr 8.

State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China School of Stomatology, Sichuan University, Chengdu 610041, China; Department of Oral Implantology, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China. Electronic address:

The present study aimed to elucidate whether extracellular signal-regulated kinases 1/2 (ERK1/2) and p38 mitogen-activated protein kinases pathways participate in the transduction of mechanical stretch exerted on adipose stem cells (ASCs) into intracellular osteogenic signals, and if so whether both pathways have time-dependent feature. Rat ASCs were cultured in osteogenic medium for 72 h and assigned into three sets, namely ERK1/2 inhibitor treated set, p38 inhibitor treated set, and the control set. After inhibitor treatment, all cells were subjected to cyclic stretch(2000 με, 1 Hz) on a four-point bending mechanical loading device. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S18735061193006
Publisher Site
http://dx.doi.org/10.1016/j.scr.2019.101433DOI Listing
April 2019
1 Read

Generation of an OCT3/4 reporter cynomolgus monkey ES cell line using CRISPR/Cas9.

Stem Cell Res 2019 Apr 15;37:101439. Epub 2019 Apr 15.

Department of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Japan; Institute for the Advanced Study of Human Biology (WPI-ASHBi), Kyoto University, Japan. Electronic address:

Cynomolgus monkey ES (Cyn ES) cells can be generated in a similar manner as human ES cells. However, Cyn ES cells are difficult to maintain in an undifferentiated state by untrained researchers. For easier culture, we generated an OCT3/4-P2A tdTomato IRES Zeocin Cyn ES cell line using CRISPR/Cas9 genome editing technology. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101439DOI Listing

Generation of induced pluripotent stem cell line-NTUHi001-A from a premature ovarian failure patient with Turner's syndrome mosaicism.

Stem Cell Res 2019 Apr 2;37:101422. Epub 2019 Apr 2.

Graduate Institute of Medical Genomics and Proteomics, College of Medicine, National Taiwan University, Taipei, Taiwan; Department of Obstetrics and Gynecology, College of Medicine and the Hospital, National Taiwan University, Taipei, Taiwan. Electronic address:

Turner's syndrome (TS) is one of the main causes of premature ovarian failure (POF). However, the mechanisms underlying POF are difficult to study due to the lack of suitable disease models. Herein, we have generated a human induced pluripotent stem cell (hiPSC) line derived from the peripheral blood mononuclear cells of a female patient with Turner's syndrome mosaicism via integration-free Sendai-virus system. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101422DOI Listing
April 2019
2 Reads

Generation of induced pluripotent stem cells (iPSCs) IRMBi002-A from an Alzheimer's disease patient carrying a D694N mutation in the APP gene.

Stem Cell Res 2019 Apr 15;37:101438. Epub 2019 Apr 15.

Institute for Regenerative Medicine and Biotherapy (IRMB), Montpellier, France; Hopital St Eloi, CHU Montpellier, 80 rue augustin Fliche, 30295 Montpellier, France. Electronic address:

Induced pluripotent stem cells (iPSC) were generated from skin fibroblasts obtained from a 58 year-old woman suffering from Alzheimer's disease and carrying a D694N mutation on Amyloid precursor protein (APP). Fibroblasts were reprogrammed into iPSC using the integration-free Sendai Virus which allows the expression of the Yamanaka factors. Verification of their pluripotency was achieved by demonstrating the expression of pluripotency markers and their differentiation potential into the three primary germ layers. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101438DOI Listing

Evaluation of commonly used ectoderm markers in iPSC trilineage differentiation.

Stem Cell Res 2019 Apr 10;37:101434. Epub 2019 Apr 10.

Children's Hospital Oakland Research Institute, 5700 Martin Luther King Jr Way, Oakland, CA 94609, USA. Electronic address:

Patient-derived induced pluripotent stem cells (iPSCs) have become a promising resource for exploring genetics of complex diseases, discovering new drugs, and advancing regenerative medicine. Increasingly, laboratories are creating their own banks of iPSCs derived from diverse donors. However, there are not yet standardized guidelines for qualifying these cell lines, i. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101434DOI Listing

Reprogramming of a human induced pluripotent stem cell (iPSC) line (IBMSi012-A) from an early-onset Parkinson's disease patient harboring a homozygous p.D331Y mutation in the PLA2G6 gene.

Stem Cell Res 2019 Apr 5;37:101432. Epub 2019 Apr 5.

Institute of Molecular Medicine, National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address:

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. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101432DOI Listing
April 2019
3 Reads

Generation of the human induced pluripotent stem cell (hiPSC) line PSMi004-A from a carrier of the KCNQ1-R594Q mutation.

Stem Cell Res 2019 Mar 27;37:101431. Epub 2019 Mar 27.

Coronary Care Unit, Laboratory of Experimental Cardiology for Cell and Molecular Therapy, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; Department of Molecular Medicine, Unit of Cardiology, Università degli Studi di Pavia, Pavia, Italy; Department of Medicine, University of Cape Town, Cape Town, South Africa. Electronic address:

We generated human induced pluripotent stem cells (hiPSCs) from dermal fibroblasts of a male carrier of the heterozygous mutation c.1781 G > A p.R594Q on the KCNQ1 gene. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S18735061193006
Publisher Site
http://dx.doi.org/10.1016/j.scr.2019.101431DOI Listing
March 2019
5 Reads

Generation of an induced pluripotent stem cell (iPSC) line, DHMCi005-A, from a patient with CALFAN syndrome due to mutations in SCYL1.

Stem Cell Res 2019 Mar 22;37:101428. Epub 2019 Mar 22.

Centre for Child and Adolescent Medicine, Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg, 69120 Heidelberg, Germany. Electronic address:

Variants in SCYL1 can cause a syndrome with low γ-glutamyl-transferase cholestasis, acute liver failure, and neurodegeneration (CALFAN). The encoded protein is involved in intracellular trafficking between Golgi and ER, specific mechanisms are still to be elucidated. We reprogrammed fibroblasts of a 2 years old male patient with CALFAN Syndrome due to a homozygous nonsense variant in SCYL1 (c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101428DOI Listing
March 2019
2 Reads

Potential therapeutic roles of stem cells in ischemia-reperfusion injury.

Stem Cell Res 2019 Mar 16;37:101421. Epub 2019 Mar 16.

Department of Molecular and Cellular Physiology, Louisiana State University Health Sciences Center Shreveport, Shreveport, LA, USA. Electronic address:

Ischemia-reperfusion injury (I/RI), produced by an initial interruption of organ blood flow and its subsequent restoration, contributes significantly to the pathophysiologies of stroke, myocardial infarction, renal I/RI, intestinal I/RI and liver I/RI, which are major causes of disability (including transplant failure) and even mortality. While the restoration of blood flow is required to restore oxygen and nutrient requirements, reperfusion often triggers local and systemic inflammatory responses and subsequently elevate the ischemic insult where the duration of ischemia determines the magnitude of I/RI damage. I/RI increases vascular leakage, changes transcriptional and cell death programs, drives leukocyte entrapment and inflammation and oxidative stress in tissues. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101421DOI Listing
March 2019
2 Reads

An induced pluripotent stem cell line (TRNDi006-A) from a MPS IIIB patient carrying homozygous mutation of p.Glu153Lys in the NAGLU gene.

Stem Cell Res 2019 Mar 23;37:101427. Epub 2019 Mar 23.

National Center for Advancing Translational Sciences, National Institutes of Health, Bethesda, MD, USA. Electronic address:

Mucopolysaccharidosis type III B (MPS IIIB) is a lysosomal storage disorder caused by mutations in the NAGLU gene encoding N-acetylglucosaminidase. Here, we report the generation of a human induced pluripotent stem cell (iPSC) line from dermal fibroblasts of a MPS IIIB patient. The iPSC line has homozygous mutations of G>A transversion at nucleotide 457 of the NAGLU gene (457G>A), resulting in the substitution of lysine for glutamic acid at codon 153 (Glu153Lys). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101427DOI Listing

Induced pluripotent stem cell (iPSC) line from an epidermolysis bullosa simplex patient heterozygous for keratin 5 E475G mutation and with the Dowling Meara phenotype.

Stem Cell Res 2019 Mar 27;37:101424. Epub 2019 Mar 27.

Medical Center for Molecular Biology, Faculty of Medicine, University of Ljubljana, Slovenia. Electronic address:

We have generated MLi002-A, a new induced pluripotent stem cell (iPSC) line derived from keratinocytes of a skin punch biopsy of a female patient with the severe epidermolysis bullosa simplex Dowling-Meara phenotype and the keratin K5 E475G mutation. Keratinocytes were reprogrammed using non-integrating Sendai virus vectors, and xeno-free culture conditions were used throughout. The characterization of MLi002-A cell line consisted of molecular karyotyping, mutation screening using restriction enzyme digestion and Sanger sequencing, and testing of the pluripotency and differentiation potentials by immunofluorescence of associated markers both in vitro and in vivo. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101424DOI Listing
March 2019
2 Reads

L-WRN conditioned medium for gastrointestinal epithelial stem cell culture shows replicable batch-to-batch activity levels across multiple research teams.

Stem Cell Res 2019 Mar 27;37:101430. Epub 2019 Mar 27.

Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110, USA. Electronic address:

Conditioned medium (CM) derived from engineered cells often facilitates the cost-effective culture of a variety of stem cells. Growing emphasis on the importance of rigor and reproducibility in lab-based science requires development of best practices approaches, including quality control procedures for the assessment of CM batches to ensure reliable interpretation and reproducibility. Here, we tested activity level variations of L-WRN CM, which is produced from an L cell line engineered to secrete Wnt3a, R spondin 3, and Noggin into a single CM that is widely used for gastrointestinal stem cell culture. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101430DOI Listing
March 2019
1 Read

Alterations in genetic and protein content of swine adipose tissue-derived mesenchymal stem cells in the metabolic syndrome.

Stem Cell Res 2019 Mar 18;37:101423. Epub 2019 Mar 18.

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, United States of America; Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, United States of America. Electronic address:

Introduction: Mesenchymal stem cells (MSCs) possess endogenous reparative properties and may serve as an exogenous therapeutic intervention in patients with chronic kidney disease. Cardiovascular risk factors clustering in the metabolic syndrome (MetS) might adversely affect cellular properties. To test the hypothesis that Mets interferes with MSC characteristics, we performed comprehensive comparison of the mRNA, microRNA, and protein content of MSCs isolated from Lean and MetS pigs. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101423DOI Listing
March 2019
2 Reads
3.693 Impact Factor

The in vivo timeline of differentiation of engrafted human neural progenitor cells.

Stem Cell Res 2019 Mar 25;37:101429. Epub 2019 Mar 25.

In-vivo-NMR Laboratory, Max Planck Institute for Metabolism Research, Gleueler Strasse 50, 50931 Cologne, Germany; Department of Neurology, University Hospital Cologne, Kerpener Strasse 62, 50937 Cologne, Germany. Electronic address:

Understanding the individual timeline of stem cell differentiation in vivo is critical for evaluating stem cell properties in animal models. However, with conventional ex vivo techniques, such as histology, the individual timeline of differentiation is not accessible. Therefore, we designed lentiviral plasmids with cell-specific promoters to control the expression of bioluminescence and fluorescence imaging reporters. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101429DOI Listing
March 2019
1 Read

Generation of an induced pluripotent stem cell (iPSC) line from a 42-year-old adult cerebral type X-linked adrenoleukodystrophy (X-ALD) patient.

Stem Cell Res 2019 Apr 21;36:101425. Epub 2019 Mar 21.

Department of Biotechnology, College of Life Science and Biotechnology, Korea University, 145 Anam-ro, Seongbuk-gu, Seoul 02841, Republic of Korea; Department of Pediatrics, Korea University College of Medicine, Guro Hospital, 97 Gurodong-gil, Guro-gu, Seoul 08308, Republic of Korea. Electronic address:

X-linked Adrenoleukodystrophy (X-ALD) is a neuro-metabolic disorder that is caused by malfunction of a peroxisomal transporter protein, adenosine ATP-binding cassette transporter superfamily D member 1 (ABCD1). We established an induced pluripotent stem cell (iPSC) line from a 42-year-old male X-ALD patient-derived dermal fibroblasts with Sendai virus-mediated reprogramming. Established iPSCs stably expanded, expressed genes of pluripotency, and maintained normal karyotype. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S18735061193005
Publisher Site
http://dx.doi.org/10.1016/j.scr.2019.101425DOI Listing
April 2019
4 Reads

Generation and characterization of the human iPSC line CABi001-A from a patient with retinitis pigmentosa caused by a novel mutation in PRPF31 gene.

Stem Cell Res 2019 Apr 20;36:101426. Epub 2019 Mar 20.

Department of Regeneration and Cell Therapy, Andalusian Molecular Biology and Regenerative Medicine Centre (CABIMER), Avda. Americo Vespucio, 24, 41092 Seville, Spain.

PRPF31 gene codes for a ubiquitously expressed splicing factor but mutations affect exclusively the retina, producing the progressive death of photoreceptor cells. We have identified a novel PRPF31 mutation in a patient with autosomal dominant retinitis pigmentosa. A blood sample was obtained and mononuclear cells were reprogrammed using the non-integrative Sendai virus to generate the cell line CABi001-A. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101426DOI Listing

Generation of two induced pluripotent stem cell lines from a patient with compound heterozygous mutations in the USH2A gene.

Stem Cell Res 2019 Apr 16;36:101420. Epub 2019 Mar 16.

Centre for Ophthalmology and Visual Sciences, The University of Western Australia, Nedlands, Western Australia, Australia; Lions Eye Institute Australia, Nedlands, Western Australia, Australia; Department of Ophthalmology, Royal Perth Hospital, Perth, Western Australia, Australia. Electronic address:

The human iPSC lines LEIi010-A and LEIi010-B were generated from the dermal fibroblasts of a patient with Usher syndrome using episomal plasmids containing OCT4, SOX2, KLF4, L-MYC, LIN28, mir302/367 microRNA and shRNA for p53. These iPSC lines carry compound heterozygous mutations (c.949C > A and c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101420DOI Listing
April 2019
2 Reads

Generation of 2 iPSC clones from a patient with DNAJC12 deficiency: DHMCi003-A and DHMCi003-B.

Stem Cell Res 2019 Apr 8;36:101402. Epub 2019 Mar 8.

Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg, Heidelberg, Germany.

Skin fibroblasts were isolated from a male patient with DNAJC12 deficiency and reprogrammed to iPSCs using the Cytotune®-iPS 2.0 Sendai Reprogramming Kit (Invitrogen). Two clones, DHMCi003-A and DHMCi003-B, were characterized for expression of pluripotency marker genes (Oct4, Nanog, Lin28, SSEA-4, TRA-1-60) and differentiated into all three germ layers using embryoid body (EB) formation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101402DOI Listing

Derivation of an aged mouse induced pluripotent stem cell line, IISHDOi005-A.

Stem Cell Res 2019 Apr 12;36:101418. Epub 2019 Mar 12.

Instituto de Investigación Sanitaria Hospital 12 de Octubre (i+12), Madrid, Spain; Centro de Investigación Biomédica en Red (CIBERER), Madrid, Spain. Electronic address:

A mouse iPSC line, IISHDOi005-A, generated from fibroblasts obtained from a mouse C57BL/6J with an age of 1 year and a half, has been obtained. For this purpose, reprogramming factors Oct3/4, Sox2, Klf4, and c-Myc were delivered using Sendai virus. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101418DOI Listing
April 2019
1 Read

Generation of the human induced pluripotent stem cell (hiPSC) line PSMi007-A from a Long QT Syndrome type 1 patient carrier of two common variants in the NOS1AP gene.

Stem Cell Res 2019 Apr 6;36:101416. Epub 2019 Mar 6.

Coronary Care Unit and Laboratory of Experimental Cardiology for Cell and Molecular Therapy, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.; Department of Molecular Medicine, Unit of Cardiology, Università degli studi di Pavia, Pavia, Italy.; Department of Medicine, University of Cape Town, Cape Town, South Africa. Electronic address:

We generated human induced pluripotent stem cells (hiPSCs) from a symptomatic Long QT Syndrome (LQTS) type 1 patient, belonging to a South African (SA) founder population segregating the heterozygous mutation c.1022C > T p.A341V on the KCNQ1 gene. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101416DOI Listing
April 2019
1 Read

Generation of two transgene-free human iPSC lines from CD133 cord blood cells.

Stem Cell Res 2019 Apr 28;36:101410. Epub 2019 Feb 28.

Inbiomed Foundation, San Sebastian, Spain. Electronic address:

We have generated two human induced pluripotent stem cell (iPSC) lines from CD133 cells isolated from umbilical cord blood (CB) of a female child using non-integrative Sendai virus. Here we describe the complete characterization of these iPSC lines: PRYDi-CB5 and PRYDi-CB40. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101410DOI Listing

Generation and characterization of a human induced pluripotent stem cell (iPSC) line (HEBHMUi001-A) from a sporadic Parkinson's disease patient.

Stem Cell Res 2019 Apr 7;36:101417. Epub 2019 Mar 7.

Hebei Medical University-National University of Ireland Galway Stem Cell Research Center, Hebei Medical University, Hebei Province 050017, China; Hebei Research Center for Stem Cell Medical Translational Engineering, Hebei Province 050017, China; Human Anatomy Department, Hebei Medical University, Hebei Province 050017, China. Electronic address:

We generated a human induced pluripotent stem cell (iPSC) line from the skin fibroblasts of a 62-year-old female patient clinically diagnosed with sporadic Parkinson's disease (PD). The generated iPSCs maintained their normal karyotype, expressed pluripotency stem cell markers, and were demonstrated to be capable of differentiating into cells representative of the three embryonic germ layers. The generated line could be used for PD modeling in order to understand the mechanisms that influence the disorder. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101417DOI Listing
April 2019
2 Reads

Generation of a human iPSC line from a patient with Marfan syndrome caused by mutation in FBN1.

Stem Cell Res 2019 Apr 27;36:101414. Epub 2019 Feb 27.

Beijing Laboratory for Cardiovascular Precision Medicine, The Key Laboratory of Remodeling-Related Cardiovascular Disease, Ministry of Education, Beijing Collaborative Innovation Center for Cardiovascular Disorders, Anzhen Hospital, Capital Medical University, Beijing 100029, China; Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing 100029, China. Electronic address:

Marfan syndrome (MFS) is a heritable connective tissue disease caused by mutations in FBN1, encoding the extracellular matrix protein fibrillin-1. In this study, we generated human induced pluripotent stem cells (iPSCs) from dermal fibroblasts of an MFS patient with the p. E2130K (c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101414DOI Listing
April 2019
2 Reads

Generation of two iPSC lines (ICGi008-A and ICGi008-B) from skin fibroblasts of a patient with early-onset Alzheimer's disease caused by London familial APP mutation (V717I).

Stem Cell Res 2019 Apr 2;36:101415. Epub 2019 Mar 2.

Federal Research Center Institute of Cytology and Genetics, the Siberian Branch of the Russian Academy of Sciences, Novosibirsk, Russia; E.N. Meshalkin National medical research center, Ministry of Health of the Russian Federation, Novosibirsk, Russia; Institute of Chemical Biology and Fundamental Medicine, the Siberian Branch of the Russian Academy of Sciences, Novosibirsk, Russia; Novosibirsk State University, Novosibirsk, Russia.

The induced pluripotent stem cell (iPSC) lines ICGi008-A and ICGi008-B were generated from dermal fibroblasts using episomal vectors expressing pluripotency factors. Dermal fibroblasts were obtained from a 55 year old male Сaucasian familial Alzheimer's disease (AD) patient carrying heterozygous V717I mutation in the APP gene. The generated iPSC lines maintained the original APP genotype, expressed pluripotency markers, exhibited a normal karyotype and retained the ability to differentiate into cell types of the three germ layers. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101415DOI Listing
April 2019
1 Read

Generation of a human induced pluripotent stem cell line, BRCi001-A, derived from a patient with mucopolysaccharidosis type I.

Stem Cell Res 2019 Apr 12;36:101406. Epub 2019 Feb 12.

iPSC-based Drug Discovery and Development Team, RIKEN BioResource Research Center, Kyoto, Japan; Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto, Japan. Electronic address:

Mucopolysaccharidosis type I (MPS I) is a rare inherited metabolic disorder caused by defects in alpha-L-iduronidase (IDUA), a lysosomal protein encoded by IDUA gene. MPS I is a progressive multisystemic disorder with a wide range of symptoms, including skeletal abnormalities and cognitive impairment, and is characterized by a wide spectrum of severity levels caused by varied mutations in IDUA. A human iPSC line was established from an attenuated MPS I (Scheie syndrome) patient carrying an IDUA gene mutation (c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101406DOI Listing
April 2019
4 Reads

Establishment of TUSMi008-A, an induced pluripotent stem cell (iPSC) line from a 76-year old Alzheimer's disease (AD) patient with PAXIP1 gene mutation.

Stem Cell Res 2019 Apr 26;36:101391. Epub 2019 Jan 26.

Stem Cell Core Facility, Translational Medical Center for Stem Cell Therapy, China; Lab of stem cell and neurodegeneration, Tongji University School of Medicine, Shanghai, China. Electronic address:

A 76-year old Alzheimer's disease (AD) female patient donated her Peripheral blood mononuclear cells (PBMC). The non-integrating episomal vector system 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. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101391DOI Listing
April 2019
1 Read

Corrigendum to "Establishment of TUSMi007-A, an induced pluripotent stem cell (iPSC) line from an 83-year old Chinese Han patient with Alzheimer's disease (AD)" [Stem Cell Research 33(2018) 265-268].

Stem Cell Res 2019 Apr 28;36:101412. Epub 2019 Feb 28.

Stem Cell Core Facility, Translational Medical Center for Stem Cell Therapy, China; Lab of stem cell and neurodegeneration, Tongji University School of Medicine, Shanghai, China. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101412DOI Listing
April 2019
1 Read

Corrigendum to Establishment of TUSMi005-A, an induced pluripotent stem cell (iPSC) line from a 32-year old Chinese Han patient with Bipolar Disorder (BD). Stem Cell Research 33(2018) 65-68.

Authors:
Jian Zhao

Stem Cell Res 2019 Apr 27;36:101413. Epub 2019 Feb 27.

Stem Cell Core Facility, Translational Medical Center for Stem Cell Therapy, Lab of stem cell and neurodegeneration, Tongji University School of Medicine, Shanghai 200120, China. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101413DOI Listing
April 2019
1 Read

Generation of induced pluripotent stem cell line, ZJUCHi002-A, from Charcot-Marie-Tooth disease type 2A (CMT2A) patient with a mutation of c.752C>T in MFN2.

Stem Cell Res 2019 Apr 20;36:101411. Epub 2019 Feb 20.

The Children's Hospital Affiliated & Key Laboratory of Diagnosis and Treatment of Neonatal Diseases of Zhejiang Province, School of Medicine, Zhejiang University, Hangzhou 310052, China; Institute of Translational Medicine, School of Medicine, Zhejiang University, Hangzhou 310020, China; Department of Neurobiology, Key Laboratory of Medical Neurobiology of Ministry of Health of China, School of Medicine, Zhejiang University, Hangzhou 310058, China. Electronic address:

The human induced pluripotent stem cell (iPSC) line ZJUCHi002-A was established from renal epithelial cells present in urine (urinary cells) collected from an 8-year-old Charcot-Marie-Tooth disease type 2A (CMT2A) patient carrying point mutation in MFN2 (c.752C > T). Urinary cells were reprogrammed by retrovirus vectors containing reprogramming factors: OCT4, SOX2, KLF4 and c-MYC. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101411DOI Listing
April 2019
2 Reads
3.693 Impact Factor

Generation of two iPS cell lines (FRIMOi003-A and FRIMOi004-A) derived from Stargardt patients carrying ABCA4 compound heterozygous mutations.

Stem Cell Res 2019 Apr 13;36:101389. Epub 2019 Feb 13.

Department of Ophthalmology, Columbia University, New York, NY, USA.

Recessive Stargardt disease (STGD1) is an autosomal recessive retinal dystrophy, caused by mutations in the retina-specific ATP-binding cassette transporter (ABCA4) gene, which plays a role as a retinaldehyde flippase in the photoreceptor outer segments. In this work, two human induced pluripotent stem cell (iPSC) lines were generated from STGD1 patients carrying compound heterozygous mutations in ABCA4. Skin fibroblasts were reprogrammed with the Yamanaka factors using a non-integrating, Sendai virus-based approach. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101389DOI Listing
April 2019
1 Read

Generation of a human induced pluripotent stem cell line (MUi010-A) from skin fibroblast of patient carrying a c.2104C>T mutation in MYH9 gene.

Stem Cell Res 2019 Apr 18;36:101397. Epub 2019 Feb 18.

Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.

Mutations in MYH9 gene is one of the major causes of inherited thrombocytopenia resulted from nonfunctional myosin-9 protein. We have generated a human induced pluripotent stem cell line MUi010-A from skin fibroblasts of a patient who had a point mutation c.2104C>T (p. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101397DOI Listing

Generation of an integration-free iPSC line(SYSUi001-A) from a sporadic Alzheimer's disease patient.

Stem Cell Res 2019 Mar 18;35:101375. Epub 2018 Dec 18.

Department of Neurology, The Sixth Affiliated Hospital of Sun Yat-Sen University, 26 Yuancun Erheng Rd, Guangzhou 510655, PR China; Guangdong Province Key Laboratory of Brain Function and Disease, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou 510080, PR China; Shenzhen Research Institute of Sun Yat-Sen University, Southern District of Nanshan Science Park, Shenzhen 518019, PR China. Electronic address:

Human iPSC line, iPSC-ADM01(SYSUi001-A), was generated from a 70-year-old male patient with sporadic Alzheimer's disease, using non-integrative reprogramming method. This cell line shows pluripotency both in vitro and in vivo, and has a normal karyotype. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2018.101375DOI Listing

Generation of an induced pluripotent stem cell line (TRNDi005-A) from a Mucopolysaccharidosis Type IVA (MPS IVA) patient carrying compound heterozygous p.R61W and p.WT405del mutations in the GALNS gene.

Stem Cell Res 2019 Apr 15;36:101408. Epub 2019 Feb 15.

National Center for Advancing Translational Sciences, National Institutes of Health, Bethesda, MD, USA. Electronic address:

Mucopolysaccharidosis type IVA (MPS IVA) is a rare genetic disease caused by mutations in the GALNS gene and is inherited in an autosomal recessive manner. GALNS encodes N-acetylgalactosamine-6-sulfatase that breaks down certain complex carbohydrates known as glycosaminoglycans (GAGs). Deficiency in this enzyme causes accumulation of GAGs in lysosomes of body tissues. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101408DOI Listing

Generation of a human Charcot-Marie-Tooth disease type 1B (CMT1B) iPSC line, ZJUCHi001-A, with a mutation of c.292C>T in MPZ.

Stem Cell Res 2019 Mar 14;35:101407. Epub 2019 Feb 14.

The Children's Hospital Affiliated, Key Laboratory of Diagnosis and Treatment of Neonatal Diseases of Zhejiang Province, School of Medicine, Zhejiang University, Hangzhou 310052, China; Institute of Translational Medicine, School of Medicine, Zhejiang University, Hangzhou 310020, China; Department of Neurobiology, Key Laboratory of Medical Neurobiology of Ministry of Health of China, School of Medicine, Zhejiang University, Hangzhou 310058, China. Electronic address:

The human iPSC cell line ZJUCHi001-A was established from renal epithelial cells present in urine (urinary cells) harvested from a 2-year-old Charcot-Marie-Tooth disease type 1B (CMT1B) patient carrying point mutation in MPZ (c.292C>T). Urinary cells were reprogrammed by retrovirus vectors containing reprogramming factors: OCT4, SOX2, KLF4 and c-MYC. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S18735061193003
Publisher Site
http://dx.doi.org/10.1016/j.scr.2019.101407DOI Listing
March 2019
7 Reads
3.693 Impact Factor

Permeability analyses and three dimensional imaging of interferon gamma-induced barrier disintegration in intestinal organoids.

Stem Cell Res 2019 Mar 7;35:101383. Epub 2019 Feb 7.

Division of Molecular and Experimental Surgery, Department of Surgery, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Translational Research Center, 91054 Erlangen, Germany; Optical Imaging Centre Erlangen (OICE), Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg, 91052 Erlangen, Germany.

The aberrant regulation of the epithelial barrier integrity is involved in many diseases of the digestive tract, including inflammatory bowel diseases and colorectal cancer. Intestinal epithelial cell organoid cultures provide new perspectives for analyses of the intestinal barrier in vitro. However, established methods of barrier function analyses from two dimensional cultures have to be adjusted to the analysis of three dimensional organoid structures. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101383DOI Listing
March 2019
3.693 Impact Factor

Generation of human iPSCs from fetal prostate fibroblasts HPrF.

Stem Cell Res 2019 Mar 7;35:101405. Epub 2019 Feb 7.

Department of Cytokinetics, Institute of Biophysics of the Czech Academy of Sciences, Brno, Czech Republic; International Clinical Research Center, St. Anne's University Hospital Brno, Brno, Czech Republic. Electronic address:

Human induced pluripotent stem cell line was generated from commercially available primary human prostate fibroblasts HPrF derived from a fetus, aged 18-24 weeks of gestation. The fibroblast cell line was reprogrammed with Yamanaka factors (OCT4, SOX2, c-MYC, KLF4) using CytoTune™-iPS 2.0 Sendai Reprogramming Kit. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101405DOI Listing

Generation of induced pluripotent stem cell line (ZZUi0012-A) from a patient with Fahr's disease caused by a novel mutation in SLC20A2 gene.

Stem Cell Res 2019 Mar 28;35:101395. Epub 2019 Jan 28.

Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China. Electronic address:

Several SLC20A2 mutations have been implicated as potential causes of Fahr's disease, a subtype of primary familial brain calcification (PFBC), but very few patient-derived induced pluripotent stem cell (iPSC) models have been established. We have identified a novel SLC20A2 mutation in a family with Fahr's disease. We subsequently obtained dermal fibroblasts from a patient in this family. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101395DOI Listing
March 2019
4 Reads
3.693 Impact Factor

Generation of an iPSC line from a patient with infantile liver failure syndrome 2 due to mutations in NBAS: DHMCi004-A.

Stem Cell Res 2019 Mar 11;35:101398. Epub 2019 Feb 11.

Centre for Child and Adolescent Medicine, Department of General Pediatrics, Division of Neuropediatrics and Metabolic Medicine, University Hospital Heidelberg, 69120 Heidelberg, Germany. Electronic address:

Fibroblasts of a patient with Infantile Liver Failure Syndrome 2 (OMIM #616483) due to a homozygous missense variant in the neuroblastoma amplified sequence gene (NBAS; c.[2708T>G]; c.[2708T>G]/p. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101398DOI Listing
March 2019
1 Read

GMP-compatible manufacturing of three iPS cell lines from human peripheral blood.

Stem Cell Res 2019 Mar 11;35:101394. Epub 2019 Feb 11.

Leibniz Research Laboratories for Biotechnology and Artificial Organs (LEBAO), REBIRTH Cluster of Excellence, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany.

The utilization of human induced pluripotent stem cells (hiPSCs) for disease modeling and drug discovery is already reality, and several first-in-man-applications as cellular therapeutics have been initiated. Implementation of good manufacturing practice (GMP)-compliant protocols for the generation of hiPSC lines is crucial to increase the application safety as well as to fulfil the legal requirements for clinical trials approval. Here we describe the development of a GMP-compatible protocol for the reprogramming of CD34 hematopoietic stem cells from peripheral blood (CD34 PBHSC) into hiPSCs using Sendai virus-based reprogramming vectors. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101394DOI Listing
March 2019
4 Reads

Generation of two isogenic iPSC lines with either a heterozygous or a homozygous E280A mutation in the PSEN1 gene.

Stem Cell Res 2019 Mar 7;35:101403. Epub 2019 Feb 7.

Bioneer A/S, Kogle Alle 2, 2970 Hørsholm, Denmark. Electronic address:

Alzheimer's disease (AD) is the most common form of dementia. Mutations in the gene PSEN1 encoding Presenilin1 are known to cause familial forms of AD with early age of onset. The most common mutation in the PSEN1 gene is the E280A mutation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101403DOI Listing
March 2019
1 Read

Constitutive activation of Notch2 signalling confers chemoresistance to neural stem cells via transactivation of fibroblast growth factor receptor-1.

Stem Cell Res 2019 Mar 7;35:101390. Epub 2019 Feb 7.

Department of Biomedicine, Pharmazentrum, University of Basel, 4056 Basel, Switzerland. Electronic address:

Notch signalling regulates neural stem cell (NSC) proliferation, differentiation and survival for the correct development and functioning of the central nervous system. Overactive Notch2 signalling has been associated with poor prognosis of aggressive brain tumours, such as glioblastoma multiforme (GBM). We recently reported that constitutive expression of the Notch2 intracellular domain (N2ICD) enhances proliferation and gliogenesis in NSCs. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101390DOI Listing
March 2019
2 Reads

Generation of a human induced pluripotent stem cell line (BIHi002-A) from a patient with CLCN7-related infantile malignant autosomal recessive osteopetrosis.

Stem Cell Res 2019 Mar 26;35:101367. Epub 2018 Dec 26.

Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt - Universität zu Berlin, Berlin Institute of Health, Berlin-Brandenburg Center for Regenerative Therapies, Berlin, Germany; Berlin Institute of Health (BIH), Berlin, Germany.

Autosomal recessive osteopetrosis (ARO) is a genetic bone disease that can be caused by mutations in the CLCN7 gene preventing osteoclast-mediated bone resorption. We generated a human induced pluripotent stem cell (hiPSC) line, BIHi002-A, from peripheral blood mononuclear cells of an ARO patient carrying the CLCN7 mutations c.875G>A and c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2018.101367DOI Listing
March 2019
3 Reads

Cymerus™ iPSC-MSCs significantly prolong survival in a pre-clinical, humanized mouse model of Graft-vs-host disease.

Stem Cell Res 2019 Mar 1;35:101401. Epub 2019 Feb 1.

Graduate Program in Molecular and Cellular Biology, University of Massachusetts Amherst, Amherst, MA 01003, United States; Department of Veterinary & Animal Sciences, University of Massachusetts Amherst, Amherst, MA 01003, United States. Electronic address:

The immune-mediated tissue destruction of graft-vs-host disease (GvHD) remains a major barrier to greater use of hematopoietic stem cell transplantation (HSCT). Mesenchymal stem cells (MSCs) have intrinsic immunosuppressive qualities and are being actively investigated as a therapeutic strategy for treating GvHD. We characterized Cymerus™ MSCs, which are derived from adult, induced pluripotent stem cells (iPSCs), and show they display surface markers and tri-lineage differentiation consistent with MSCs isolated from bone marrow (BM). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101401DOI Listing
March 2019
2 Reads
3.693 Impact Factor

Corrigendum to "Generation of induced pluripotent stem cells from a patient with X-linked juvenile retinoschisis" [Stem Cell Res. 29(2018): 152-156].

Stem Cell Res 2019 Jan;34:101388

Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan; Genomic Research Center, Academia Sinica, Taipei, Taiwan; Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101388DOI Listing
January 2019
1 Read
3.693 Impact Factor

Differentiation and isolation of iPSC-derived remodeling ductal plate-like cells by use of an AQP1-GFP reporter human iPSC line.

Stem Cell Res 2019 Mar 31;35:101400. Epub 2019 Jan 31.

Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto, Japan. Electronic address:

Cholangiocytes are the epithelial cells that line bile ducts, and ductal plate malformation is a developmental anomaly of bile ducts that causes severe congenital biliary disorders. However, because of a lack of specific marker genes, methods for the stepwise differentiation and isolation of human induced pluripotent stem cell (hiPSC)-derived cholangiocyte progenitors at ductal plate stages have not been established. We herein generated an AQP1-GFP reporter hiPSC line and developed a combination treatment with transforming growth factor (TGF) β2 and epidermal growth factor (EGF) to induce hiPSC-derived hepatoblasts into AQP1 cells in vitro. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101400DOI Listing

Generation of two induced pluripotent stem cell lines from skin fibroblasts of sisters carrying a c.1094C>A variation in the SCN10A gene potentially associated with small fiber neuropathy.

Stem Cell Res 2019 Mar 2;35:101396. Epub 2019 Feb 2.

Department of Neurology, University of Würzburg, Würzburg, Germany. Electronic address:

Induced pluripotent stem cells (iPSC) were derived from human dermal fibroblasts (HDF) of two siblings with small fiber neuropathy (SFN) potentially based on the same variation in SCN10A but exhibiting diverse disease phenotypes. HDF were reprogrammed using a non-integrating mRNA approach and showed robust expression of pluripotency markers. iPSC displayed no chromosomal aberrations and were differentiated into all three germ-layers. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2019.101396DOI Listing