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  • 1
    Publication Date: 2002-02-02
    Description: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Cibelli, Jose B -- Grant, Kathleen A -- Chapman, Karen B -- Cunniff, Kerrianne -- Worst, Travis -- Green, Heather L -- Walker, Stephen J -- Gutin, Philip H -- Vilner, Lucy -- Tabar, Viviane -- Dominko, Tanja -- Kane, Jeff -- Wettstein, Peter J -- Lanza, Robert P -- Studer, Lorenz -- Vrana, Kent E -- West, Michael D -- P50-AA11997/AA/NIAAA NIH HHS/ -- T32-AA07565/AA/NIAAA NIH HHS/ -- New York, N.Y. -- Science. 2002 Feb 1;295(5556):819.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Advanced Cell Technology, One Innovation Drive, Worcester, MA 01605, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/11823632" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Astrocytes/cytology ; Blastocyst/*cytology/physiology ; Cell Culture Techniques ; Cell Differentiation ; Cell Division ; Cell Line ; Cell Separation ; Cloning, Organism ; Dopamine/metabolism ; Embryo, Mammalian/*cytology ; Karyotyping ; *Macaca fascicularis ; Mice ; Mice, SCID ; Neurons/cytology ; *Parthenogenesis ; Serotonin/metabolism ; Stem Cells/*cytology/physiology ; Teratoma/pathology
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
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  • 2
    Publication Date: 2016-02-11
    Description: The enteric nervous system (ENS) is the largest component of the autonomic nervous system, with neuron numbers surpassing those present in the spinal cord. The ENS has been called the 'second brain' given its autonomy, remarkable neurotransmitter diversity and complex cytoarchitecture. Defects in ENS development are responsible for many human disorders including Hirschsprung disease (HSCR). HSCR is caused by the developmental failure of ENS progenitors to migrate into the gastrointestinal tract, particularly the distal colon. Human ENS development remains poorly understood owing to the lack of an easily accessible model system. Here we demonstrate the efficient derivation and isolation of ENS progenitors from human pluripotent stem (PS) cells, and their further differentiation into functional enteric neurons. ENS precursors derived in vitro are capable of targeted migration in the developing chick embryo and extensive colonization of the adult mouse colon. The in vivo engraftment and migration of human PS-cell-derived ENS precursors rescue disease-related mortality in HSCR mice (Ednrb(s-l/s-l)), although the mechanism of action remains unclear. Finally, EDNRB-null mutant ENS precursors enable modelling of HSCR-related migration defects, and the identification of pepstatin A as a candidate therapeutic target. Our study establishes the first, to our knowledge, human PS-cell-based platform for the study of human ENS development, and presents cell- and drug-based strategies for the treatment of HSCR.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4846424/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4846424/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Fattahi, Faranak -- Steinbeck, Julius A -- Kriks, Sonja -- Tchieu, Jason -- Zimmer, Bastian -- Kishinevsky, Sarah -- Zeltner, Nadja -- Mica, Yvonne -- El-Nachef, Wael -- Zhao, Huiyong -- de Stanchina, Elisa -- Gershon, Michael D -- Grikscheit, Tracy C -- Chen, Shuibing -- Studer, Lorenz -- DP2 DK098093-01/DK/NIDDK NIH HHS/ -- NS15547/NS/NINDS NIH HHS/ -- P30 CA008748/CA/NCI NIH HHS/ -- R01 NS015547/NS/NINDS NIH HHS/ -- England -- Nature. 2016 Mar 3;531(7592):105-9. doi: 10.1038/nature16951. Epub 2016 Feb 10.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉The Center for Stem Cell Biology, New York, New York 10065, USA. ; Developmental Biology Program, Sloan-Kettering Institute for Cancer Research, New York, New York 10065, USA. ; Weill Graduate School of Medical Sciences of Cornell University, New York, New York 10065, USA. ; Molecular Pharmacology Program, New York, New York 10065, USA. ; Department of Pathology and Cell Biology, Columbia University, College of Physicians and Surgeons, New York, New York 10032, USA. ; Children's Hospital Los Angeles, Pediatric Surgery, Los Angeles, California 90027, USA. ; Department of Surgery, Weill Medical College of Cornell University, New York, New York 10065, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/26863197" target="_blank"〉PubMed〈/a〉
    Keywords: Aging ; Animals ; Cell Differentiation ; Cell Line ; *Cell Lineage ; Cell Movement ; Cell Separation ; *Cell- and Tissue-Based Therapy/methods ; Chick Embryo ; Colon/drug effects/pathology ; Disease Models, Animal ; Drug Discovery/*methods ; Enteric Nervous System/*pathology ; Female ; Gastrointestinal Tract/drug effects/pathology ; Hirschsprung Disease/*drug therapy/*pathology/therapy ; Humans ; Male ; Mice ; Neurons/drug effects/*pathology ; Pepstatins/metabolism ; Pluripotent Stem Cells/pathology ; Receptor, Endothelin B/metabolism ; Signal Transduction
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 3
    Publication Date: 2009-08-21
    Description: The isolation of human induced pluripotent stem cells (iPSCs) offers a new strategy for modelling human disease. Recent studies have reported the derivation and differentiation of disease-specific human iPSCs. However, a key challenge in the field is the demonstration of disease-related phenotypes and the ability to model pathogenesis and treatment of disease in iPSCs. Familial dysautonomia (FD) is a rare but fatal peripheral neuropathy, caused by a point mutation in the IKBKAP gene involved in transcriptional elongation. The disease is characterized by the depletion of autonomic and sensory neurons. The specificity to the peripheral nervous system and the mechanism of neuron loss in FD are poorly understood owing to the lack of an appropriate model system. Here we report the derivation of patient-specific FD-iPSCs and the directed differentiation into cells of all three germ layers including peripheral neurons. Gene expression analysis in purified FD-iPSC-derived lineages demonstrates tissue-specific mis-splicing of IKBKAP in vitro. Patient-specific neural crest precursors express particularly low levels of normal IKBKAP transcript, suggesting a mechanism for disease specificity. FD pathogenesis is further characterized by transcriptome analysis and cell-based assays revealing marked defects in neurogenic differentiation and migration behaviour. Furthermore, we use FD-iPSCs for validating the potency of candidate drugs in reversing aberrant splicing and ameliorating neuronal differentiation and migration. Our study illustrates the promise of iPSC technology for gaining new insights into human disease pathogenesis and treatment.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2784695/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2784695/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Lee, Gabsang -- Papapetrou, Eirini P -- Kim, Hyesoo -- Chambers, Stuart M -- Tomishima, Mark J -- Fasano, Christopher A -- Ganat, Yosif M -- Menon, Jayanthi -- Shimizu, Fumiko -- Viale, Agnes -- Tabar, Viviane -- Sadelain, Michel -- Studer, Lorenz -- R01 NS052671/NS/NINDS NIH HHS/ -- R01 NS052671-03/NS/NINDS NIH HHS/ -- England -- Nature. 2009 Sep 17;461(7262):402-6. doi: 10.1038/nature08320. Epub 2009 Aug 19.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Developmental Biology Program, Sloan-Kettering Institute, 1275 York Ave, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19693009" target="_blank"〉PubMed〈/a〉
    Keywords: Adolescent ; Alternative Splicing/drug effects/genetics ; Animals ; Carrier Proteins/genetics ; Cell Dedifferentiation ; Cell Differentiation ; Cell Lineage ; Cell Movement ; Cells, Cultured ; Child ; Dysautonomia, Familial/drug therapy/genetics/*pathology/*therapy ; Female ; Fibroblasts/cytology/metabolism ; Gene Expression Profiling ; Humans ; Kinetin/pharmacology/therapeutic use ; Male ; Mice ; *Models, Biological ; Neural Crest/cytology/drug effects ; Organ Specificity ; Phenotype ; Pluripotent Stem Cells/cytology/drug effects/*metabolism/*transplantation
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 4
    Publication Date: 2011-11-08
    Description: Human pluripotent stem cells (PSCs) are a promising source of cells for applications in regenerative medicine. Directed differentiation of PSCs into specialized cells such as spinal motoneurons or midbrain dopamine (DA) neurons has been achieved. However, the effective use of PSCs for cell therapy has lagged behind. Whereas mouse PSC-derived DA neurons have shown efficacy in models of Parkinson's disease, DA neurons from human PSCs generally show poor in vivo performance. There are also considerable safety concerns for PSCs related to their potential for teratoma formation or neural overgrowth. Here we present a novel floor-plate-based strategy for the derivation of human DA neurons that efficiently engraft in vivo, suggesting that past failures were due to incomplete specification rather than a specific vulnerability of the cells. Midbrain floor-plate precursors are derived from PSCs 11 days after exposure to small molecule activators of sonic hedgehog (SHH) and canonical WNT signalling. Engraftable midbrain DA neurons are obtained by day 25 and can be maintained in vitro for several months. Extensive molecular profiling, biochemical and electrophysiological data define developmental progression and confirm identity of PSC-derived midbrain DA neurons. In vivo survival and function is demonstrated in Parkinson's disease models using three host species. Long-term engraftment in 6-hydroxy-dopamine-lesioned mice and rats demonstrates robust survival of midbrain DA neurons derived from human embryonic stem (ES) cells, complete restoration of amphetamine-induced rotation behaviour and improvements in tests of forelimb use and akinesia. Finally, scalability is demonstrated by transplantation into parkinsonian monkeys. Excellent DA neuron survival, function and lack of neural overgrowth in the three animal models indicate promise for the development of cell-based therapies in Parkinson's disease.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3245796/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3245796/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Kriks, Sonja -- Shim, Jae-Won -- Piao, Jinghua -- Ganat, Yosif M -- Wakeman, Dustin R -- Xie, Zhong -- Carrillo-Reid, Luis -- Auyeung, Gordon -- Antonacci, Chris -- Buch, Amanda -- Yang, Lichuan -- Beal, M Flint -- Surmeier, D James -- Kordower, Jeffrey H -- Tabar, Viviane -- Studer, Lorenz -- NS052671/NS/NINDS NIH HHS/ -- P50 NS047085/NS/NINDS NIH HHS/ -- P50 NS071669/NS/NINDS NIH HHS/ -- P50 NS071669-03/NS/NINDS NIH HHS/ -- England -- Nature. 2011 Nov 6;480(7378):547-51. doi: 10.1038/nature10648.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Center for Stem Cell Biology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, New York 10065, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/22056989" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; *Brain Tissue Transplantation ; Cell Differentiation ; Cell Line ; Cell Survival ; Dopaminergic Neurons/*cytology/*transplantation ; Embryonic Stem Cells/*cytology ; Female ; Humans ; Macaca mulatta ; Mesencephalon/cytology ; Mice ; Mice, Inbred NOD ; Mice, SCID ; Parkinson Disease/*therapy ; Rats ; Rats, Sprague-Dawley
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 5
    Publication Date: 2012-10-30
    Description: In the course of primary infection with herpes simplex virus 1 (HSV-1), children with inborn errors of toll-like receptor 3 (TLR3) immunity are prone to HSV-1 encephalitis (HSE). We tested the hypothesis that the pathogenesis of HSE involves non-haematopoietic CNS-resident cells. We derived induced pluripotent stem cells (iPSCs) from the dermal fibroblasts of TLR3- and UNC-93B-deficient patients and from controls. These iPSCs were differentiated into highly purified populations of neural stem cells (NSCs), neurons, astrocytes and oligodendrocytes. The induction of interferon-beta (IFN-beta) and/or IFN-lambda1 in response to stimulation by the dsRNA analogue polyinosinic:polycytidylic acid (poly(I:C)) was dependent on TLR3 and UNC-93B in all cells tested. However, the induction of IFN-beta and IFN-lambda1 in response to HSV-1 infection was impaired selectively in UNC-93B-deficient neurons and oligodendrocytes. These cells were also much more susceptible to HSV-1 infection than control cells, whereas UNC-93B-deficient NSCs and astrocytes were not. TLR3-deficient neurons were also found to be susceptible to HSV-1 infection. The rescue of UNC-93B- and TLR3-deficient cells with the corresponding wild-type allele showed that the genetic defect was the cause of the poly(I:C) and HSV-1 phenotypes. The viral infection phenotype was rescued further by treatment with exogenous IFN-alpha or IFN-beta ( IFN-alpha/beta) but not IFN-lambda1. Thus, impaired TLR3- and UNC-93B-dependent IFN-alpha/beta intrinsic immunity to HSV-1 in the CNS, in neurons and oligodendrocytes in particular, may underlie the pathogenesis of HSE in children with TLR3-pathway deficiencies.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3527075/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3527075/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Lafaille, Fabien G -- Pessach, Itai M -- Zhang, Shen-Ying -- Ciancanelli, Michael J -- Herman, Melina -- Abhyankar, Avinash -- Ying, Shui-Wang -- Keros, Sotirios -- Goldstein, Peter A -- Mostoslavsky, Gustavo -- Ordovas-Montanes, Jose -- Jouanguy, Emmanuelle -- Plancoulaine, Sabine -- Tu, Edmund -- Elkabetz, Yechiel -- Al-Muhsen, Saleh -- Tardieu, Marc -- Schlaeger, Thorsten M -- Daley, George Q -- Abel, Laurent -- Casanova, Jean-Laurent -- Studer, Lorenz -- Notarangelo, Luigi D -- 1R01NS066390/NS/NINDS NIH HHS/ -- 1R03AI0883502-01/AI/NIAID NIH HHS/ -- 5R01NS072381-02/NS/NINDS NIH HHS/ -- 8UL1TR000043/TR/NCATS NIH HHS/ -- K12 NS066274/NS/NINDS NIH HHS/ -- R01 NS066390/NS/NINDS NIH HHS/ -- R01 NS072381/NS/NINDS NIH HHS/ -- R03 AI088352/AI/NIAID NIH HHS/ -- UL1 TR000043/TR/NCATS NIH HHS/ -- England -- Nature. 2012 Nov 29;491(7426):769-73. doi: 10.1038/nature11583. Epub 2012 Oct 28.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Center for Stem Cell Biology, Sloan-Kettering Institute for Cancer Research, New York, New York 10065, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/23103873" target="_blank"〉PubMed〈/a〉
    Keywords: Astrocytes/immunology/virology ; Biomarkers ; Cell Differentiation ; Cell Lineage ; Cell Separation ; Cells, Cultured ; Central Nervous System/cytology/immunology/*pathology/virology ; Child ; Disease Susceptibility ; Encephalitis, Herpes Simplex/immunology/metabolism/pathology/virology ; Herpesvirus 1, Human/*immunology/pathogenicity ; Humans ; Immunity, Innate ; Induced Pluripotent Stem Cells/*cytology/virology ; Interferons/immunology ; Membrane Transport Proteins/deficiency/genetics ; Neural Stem Cells/immunology/virology ; Neurons/immunology/pathology/virology ; Oligodendroglia/immunology/pathology/virology ; Toll-Like Receptor 3/*deficiency/genetics
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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