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Stem Cell Reports

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https://www.readbyqxmd.com/read/30416049/patient-specific-ipsc-derived-endothelial-cells-provide-long-term-phenotypic-correction-of-hemophilia-a
#1
Cristina Olgasi, Maria Talmon, Simone Merlin, Alessia Cucci, Yvonne Richaud-Patin, Gabriella Ranaldo, Donato Colangelo, Federica Di Scipio, Giovanni N Berta, Chiara Borsotti, Federica Valeri, Francesco Faraldi, Maria Prat, Maria Messina, Piercarla Schinco, Angelo Lombardo, Angel Raya, Antonia Follenzi
We generated patient-specific disease-free induced pluripotent stem cells (iPSCs) from peripheral blood CD34+ cells and differentiated them into functional endothelial cells (ECs) secreting factor VIII (FVIII) for gene and cell therapy approaches to cure hemophilia A (HA), an X-linked bleeding disorder caused by F8 mutations. iPSCs were transduced with a lentiviral vector carrying FVIII transgene driven by an endothelial-specific promoter (VEC) and differentiated into bona fide ECs using an optimized protocol...
November 3, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30416050/excitable-adult-generated-gabaergic-neurons-acquire-functional-innervation-in-the-cortex-after-stroke
#2
Timal S Kannangara, Anthony Carter, Yingben Xue, Jagroop S Dhaliwal, Jean-Claude Béïque, Diane C Lagace
Ischemic stroke enhances the proliferation of adult-generated precursor cells that ectopically migrate toward the infarct. Studies have correlated precursor cell proliferation and subsequent adult neurogenesis with enhanced stroke recovery, yet it remains unclear whether stroke can generate new neurons capable of functional integration into the injured cortex. Here, using single and bitransgenic reporter mice, we identify spatial and temporal features of a multilineage cellular response to focal ischemia. We reveal that a small population of stroke-induced immature neurons accumulate within the peri-infarct region of the adult sensorimotor cortex, exhibit voltage-dependent conductances, fire action potentials, express GABAergic markers, and receive sparse GABAergic synaptic inputs...
October 30, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30416051/atrial-like-engineered-heart-tissue-an-in-vitro-model-of-the-human-atrium
#3
Marta Lemme, Bärbel M Ulmer, Marc D Lemoine, Antonia T L Zech, Frederik Flenner, Ursula Ravens, Hermann Reichenspurner, Miriam Rol-Garcia, Godfrey Smith, Arne Hansen, Torsten Christ, Thomas Eschenhagen
Cardiomyocytes (CMs) generated from human induced pluripotent stem cells (hiPSCs) are under investigation for their suitability as human models in preclinical drug development. Antiarrhythmic drug development focuses on atrial biology for the treatment of atrial fibrillation. Here we used recent retinoic acid-based protocols to generate atrial CMs from hiPSCs and establish right atrial engineered heart tissue (RA-EHT) as a 3D model of human atrium. EHT from standard protocol-derived hiPSC-CMs (Ctrl-EHT) and intact human muscle strips served as comparators...
October 27, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30416048/the-ubiquitin-proteasome-system-is-indispensable-for-the-maintenance-of-muscle-stem-cells
#4
Yasuo Kitajima, Naoki Suzuki, Aki Nunomiya, Shion Osana, Kiyoshi Yoshioka, Yoshitaka Tashiro, Ryosuke Takahashi, Yusuke Ono, Masashi Aoki, Ryoichi Nagatomi
Adult muscle stem cells (satellite cells) are required for adult skeletal muscle regeneration. A proper balance between quiescence, proliferation, and differentiation is essential for the maintenance of the satellite cell pool and their regenerative function. Although the ubiquitin-proteasome is required for most protein degradation in mammalian cells, how its dysfunction affects tissue stem cells remains unclear. Here, we investigated the function of the proteasome in satellite cells using mice lacking the crucial proteasomal component, Rpt3...
October 25, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30428387/intestinal-stem-cells-exhibit-conditional-circadian-clock-function
#5
Kathyani Parasram, Nathaniel Bernardon, Maha Hammoud, Hanna Chang, Li He, Norbert Perrimon, Phillip Karpowicz
The circadian clock is a molecular pacemaker that produces 24-hr physiological cycles known as circadian rhythms. How the clock regulates stem cells is an emerging area of research with many outstanding questions. We tested clock function in vivo at the single cell resolution in the Drosophila intestine, a tissue that is exquisitely sensitive to environmental cues and has circadian rhythms in regeneration. Our results indicate that circadian clocks function in intestinal stem cells and enterocytes but are downregulated during enteroendocrine cell differentiation...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30428386/a-prospective-analysis-of-human-leukemogenesis
#6
REVIEW
Connie J Eaves
Decades of lack of progress in treating many fatal malignancies of the blood-forming system is commanding interest in new approaches. Targeting early events in the leukemogenic process has long been recognized as likely to offer the information required for these diseases. Analysis of the representation of different mutations in the leukemic cells from individual patients offers a retrospective method to infer their sequence of acquisition and associated subclonal dynamics. An alternative, prospective approach is to exploit strategies for recreating human leukemia de novo using defined genetic methods...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30428385/living-with-keratinocytes
#7
REVIEW
Graziella Pellegrini, Michele De Luca
A feature distinguishing human hematopoietic and epithelial stem cells from other equally fascinating stem cells is perhaps their easier translation into a clinical setting. We have devoted nearly our entire scientific career in trying to turn our understanding of epithelial stem cell biology into something that could help people suffering from virtually untreatable diseases of squamous epithelia. We have done that as a team, together with our numerous students, postdocs, technicians and valuable collaborators, clinicians, regulators, and, lately, industrial partners...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30428384/tackling-ethical-challenges-of-premature-delivery-of-stem-cell-based-therapies-isscr-2018-annual-meeting-focus-session-report
#8
Jeremy Sugarman, Roger A Barker, Ian Kerridge, Tamra Lysaght, Graziella Pellegrini, Douglas Sipp, Claire Tanner
Clinical uses of unproven stem cell-based interventions abound, yet many patients may be harmed by receiving them, raising complex ethical, economic, and societal concerns. Regulators, scientists, clinicians, professional societies, and patient advocacy groups need to collaboratively articulate expectations related to the proper development and delivery of stem cell-based therapies.
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30409508/human-induced-pluripotent-stem-cell-derived-astrocytes-are-differentially-activated-by-multiple-sclerosis-associated-cytokines
#9
Sylvain Perriot, Amandine Mathias, Guillaume Perriard, Mathieu Canales, Nils Jonkmans, Nicolas Merienne, Cécile Meunier, Lina El Kassar, Anselme L Perrier, David-Axel Laplaud, Myriam Schluep, Nicole Déglon, Renaud Du Pasquier
Recent studies highlighted the importance of astrocytes in neuroinflammatory diseases, interacting closely with other CNS cells but also with the immune system. However, due to the difficulty in obtaining human astrocytes, their role in these pathologies is still poorly characterized. Here, we develop a serum-free protocol to differentiate human induced pluripotent stem cells (hiPSCs) into astrocytes. Gene expression and functional assays show that our protocol consistently yields a highly enriched population of resting mature astrocytes across the 13 hiPSC lines differentiated...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30392977/an-mtor-signaling-modulator-suppressed-heterotopic-ossification-of-fibrodysplasia-ossificans-progressiva
#10
Kyosuke Hino, Chengzhu Zhao, Kazuhiko Horigome, Megumi Nishio, Yasue Okanishi, Sanae Nagata, Shingo Komura, Yasuhiro Yamada, Junya Toguchida, Akira Ohta, Makoto Ikeya
Fibrodysplasia ossificans progressiva (FOP) is a rare and intractable disorder characterized by extraskeletal bone formation through endochondral ossification. FOP patients harbor gain-of-function mutations in ACVR1 (FOP-ACVR1), a type I receptor for bone morphogenetic proteins. Despite numerous studies, no drugs have been approved for FOP. Here, we developed a high-throughput screening (HTS) system focused on the constitutive activation of FOP-ACVR1 by utilizing a chondrogenic ATDC5 cell line that stably expresses FOP-ACVR1...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30392976/complete-disruption-of-autism-susceptibility-genes-by-gene-editing-predominantly-reduces-functional-connectivity-of-isogenic-human-neurons
#11
Eric Deneault, Sean H White, Deivid C Rodrigues, P Joel Ross, Muhammad Faheem, Kirill Zaslavsky, Zhuozhi Wang, Roumiana Alexandrova, Giovanna Pellecchia, Wei Wei, Alina Piekna, Gaganjot Kaur, Jennifer L Howe, Vickie Kwan, Bhooma Thiruvahindrapuram, Susan Walker, Anath C Lionel, Peter Pasceri, Daniele Merico, Ryan K C Yuen, Karun K Singh, James Ellis, Stephen W Scherer
Autism spectrum disorder (ASD) is phenotypically and genetically heterogeneous. We present a CRISPR gene editing strategy to insert a protein tag and premature termination sites creating an induced pluripotent stem cell (iPSC) knockout resource for functional studies of ten ASD-relevant genes (AFF2/FMR2, ANOS1, ASTN2, ATRX, CACNA1C, CHD8, DLGAP2, KCNQ2, SCN2A, TENM1). Neurogenin 2 (NGN2)-directed induction of iPSCs allowed production of excitatory neurons, and mutant proteins were not detectable. RNA sequencing revealed convergence of several neuronal networks...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30392975/isogenic-pairs-of-hipsc-cms-with-hypertrophic-cardiomyopathy-lvnc-associated-actc1-e99k-mutation-unveil-differential-functional-deficits
#12
James G W Smith, Thomas Owen, Jamie R Bhagwan, Diogo Mosqueira, Elizabeth Scott, Ingra Mannhardt, Asha Patel, Roberto Barriales-Villa, Lorenzo Monserrat, Arne Hansen, Thomas Eschenhagen, Sian E Harding, Steve Marston, Chris Denning
Hypertrophic cardiomyopathy (HCM) is a primary disorder of contractility in heart muscle. To gain mechanistic insight and guide pharmacological rescue, this study models HCM using isogenic pairs of human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) carrying the E99K-ACTC1 cardiac actin mutation. In both 3D engineered heart tissues and 2D monolayers, arrhythmogenesis was evident in all E99K-ACTC1 hiPSC-CMs. Aberrant phenotypes were most common in hiPSC-CMs produced from the heterozygote father...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30392974/proteinase-3-limits-the-number-of-hematopoietic-stem-and-progenitor-cells-in-murine-bone-marrow
#13
Kutay Karatepe, Haiyan Zhu, Xiaoyu Zhang, Rongxia Guo, Hiroto Kambara, Fabien Loison, Peng Liu, Hongbo Yu, Qian Ren, Xiao Luo, John Manis, Tao Cheng, Fengxia Ma, Yuanfu Xu, Hongbo R Luo
Hematopoietic stem and progenitor cells (HSPCs) undergo self-renewal and differentiation to guarantee a constant supply of short-lived blood cells. Both intrinsic and extrinsic factors determine HSPC fate, but the underlying mechanisms remain elusive. Here, we report that Proteinase 3 (PR3), a serine protease mainly confined to granulocytes, is also expressed in HSPCs. PR3 deficiency intrinsically suppressed cleavage and activation of caspase-3, leading to expansion of the bone marrow (BM) HSPC population due to decreased apoptosis...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30392973/generation-of-progesterone-responsive-endometrial-stromal-fibroblasts-from-human-induced-pluripotent-stem-cells-role-of-the-wnt-ctnnb1-pathway
#14
Kaoru Miyazaki, Matthew T Dyson, John S Coon V, Yuichi Furukawa, Bahar D Yilmaz, Tetsuo Maruyama, Serdar E Bulun
Defective endometrial stromal fibroblasts (EMSFs) contribute to uterine factor infertility, endometriosis, and endometrial cancer. Induced pluripotent stem cells (iPSCs) derived from skin or bone marrow biopsies provide a patient-specific source that can be differentiated to various cells types. Replacement of abnormal EMSFs is a potential novel therapeutic approach for endometrial disease; however, the methodology or mechanism for differentiating iPSCs to EMSFs is unknown. The uterus differentiates from the intermediate mesoderm (IM) to form coelomic epithelium (CE) followed by the Müllerian duct (MD)...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30344010/human-teratoma-derived-hematopoiesis-is-a-highly-polyclonal-process-supported-by-human-umbilical-vein-endothelial-cells
#15
Friederike Philipp, Anton Selich, Michael Rothe, Dirk Hoffmann, Susanne Rittinghausen, Michael A Morgan, Denise Klatt, Silke Glage, Stefan Lienenklaus, Vanessa Neuhaus, Katherina Sewald, Armin Braun, Axel Schambach
Hematopoietic stem cells (HSCs) ensure a life-long regeneration of the blood system and are therefore an important source for transplantation and gene therapy. The teratoma environment supports the complex development of functional HSCs from human pluripotent stem cells, which is difficult to recapitulate in culture. This model mimics various aspects of early hematopoiesis, but is restricted by the low spontaneous hematopoiesis rate. In this study, a feasible protocol for robust hematopoiesis has been elaborated...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30344009/the-scrib-paralog-lano-lrrc1-regulates-breast-cancer-stem-cell-fate-through-wnt-%C3%AE-catenin-signaling
#16
Leonor Lopez Almeida, Michael Sebbagh, François Bertucci, Pascal Finetti, Julien Wicinski, Sylvie Marchetto, Rémy Castellano, Emmanuelle Josselin, Emmanuelle Charafe-Jauffret, Christophe Ginestier, Jean-Paul Borg, Marie-Josée Santoni
Tumor initiation, progression, and therapeutic resistance have been proposed to originate from a subset of tumor cells, cancer stem cells (CSCs). However, the current understanding of the mechanisms involved in their self-renewal and tumor initiation capacity remains limited. Here, we report that expression of LANO/LRRC1, the vertebrate paralog of SCRIB tumor suppressor, is associated with a stem cell signature in normal and tumoral mammary epithelia. Through in vitro and in vivo experiments including a Lano/Lrrc1 knockout mouse model, we demonstrate its involvement in the regulation of breast CSC (bCSC) fate...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30344008/connexin-43-mediated-mitochondrial-transfer-of-ipsc-mscs-alleviates-asthma-inflammation
#17
Yin Yao, Xing-Liang Fan, Dan Jiang, Yuelin Zhang, Xin Li, Zhi-Bin Xu, Shu-Bin Fang, Sinming Chiu, Hung-Fat Tse, Qizhou Lian, Qing-Ling Fu
We previously identified an immunomodulatory role of human induced pluripotent stem cell (iPSC)-derived mesenchymal stem cells (MSCs) in asthmatic inflammation. Mitochondrial transfer from bone marrow MSCs to epithelial cells can result in the attenuation of acute lung injury in mice. However, the effects of mitochondrial transfer from iPSC-MSCs to epithelial cells in asthma and the mechanisms underlying these effects are unclear. We found that iPSC-MSC transplantation significantly reduced T helper 2 cytokines, attenuated the mitochondrial dysfunction of epithelial cells, and alleviated asthma inflammation in mice...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30344007/mitochondrial-dysregulation-and-impaired-autophagy-in-ipsc-derived-dopaminergic-neurons-of-multiple-system-atrophy
#18
Giacomo Monzio Compagnoni, Giulio Kleiner, Maura Samarani, Massimo Aureli, Gaia Faustini, Arianna Bellucci, Dario Ronchi, Andreina Bordoni, Manuela Garbellini, Sabrina Salani, Francesco Fortunato, Emanuele Frattini, Elena Abati, Christian Bergamini, Romana Fato, Silvia Tabano, Monica Miozzo, Giulia Serratto, Maria Passafaro, Michela Deleidi, Rosamaria Silipigni, Monica Nizzardo, Nereo Bresolin, Giacomo P Comi, Stefania Corti, Catarina M Quinzii, Alessio Di Fonzo
Multiple system atrophy (MSA) is a progressive neurodegenerative disease that affects several areas of the CNS, whose pathogenesis is still widely unclear and for which an effective treatment is lacking. We have generated induced pluripotent stem cell-derived dopaminergic neurons from four MSA patients and four healthy controls and from two monozygotic twins discordant for the disease. In this model, we have demonstrated an aberrant autophagic flow and a mitochondrial dysregulation involving respiratory chain activity, mitochondrial content, and CoQ10 biosynthesis...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30344006/t-type-calcium-channels-determine-the-vulnerability-of-dopaminergic-neurons-to-mitochondrial-stress-in-familial-parkinson-disease
#19
Yoshikuni Tabata, Yoichi Imaizumi, Michiko Sugawara, Tomoko Andoh-Noda, Satoe Banno, MuhChyi Chai, Takefumi Sone, Kazuto Yamazaki, Masashi Ito, Kappei Tsukahara, Hideyuki Saya, Nobutaka Hattori, Jun Kohyama, Hideyuki Okano
Parkinson disease (PD) is a progressive neurological disease caused by selective degeneration of dopaminergic (DA) neurons in the substantia nigra. Although most cases of PD are sporadic cases, familial PD provides a versatile research model for basic mechanistic insights into the pathogenesis of PD. In this study, we generated DA neurons from PARK2 patient-specific, isogenic PARK2 null and PARK6 patient-specific induced pluripotent stem cells and found that these neurons exhibited more apoptosis and greater susceptibility to rotenone-induced mitochondrial stress...
November 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30318292/phenotypic-reprogramming-of-striatal-neurons-into-dopaminergic-neuron-like-cells-in-the-adult-mouse-brain
#20
Wenze Niu, Tong Zang, Lei-Lei Wang, Yuhua Zou, Chun-Li Zhang
Neuronal subtype is largely fixed in the adult mammalian brain. Here, however, we unexpectedly reveal that adult mouse striatal neurons can be reprogrammed into dopaminergic neuron-like cells (iDALs). This in vivo phenotypic reprogramming can be promoted by a stem cell factor (SOX2), three dopaminergic neuron-enriched transcription regulators (NURR1, LMX1A, and FOXA2), and a chemical compound (valproic acid). Although the site of action of the reprogramming factors remains to be determined, immunohistochemistry and genetic lineage mappings confirm striatal neurons as the cell origin for iDALs...
November 13, 2018: Stem Cell Reports
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