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Author (up) Acab, A.; Muotri, A.R. url  doi
openurl 
  Title The Use of Induced Pluripotent Stem Cell Technology to Advance Autism Research and Treatment Type Journal Article
  Year 2015 Publication Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics Abbreviated Journal Neurotherapeutics  
  Volume 12 Issue 3 Pages 534-545  
  Keywords Autism Spectrum Disorder/*drug therapy/genetics/metabolism/*physiopathology; Drug Evaluation, Preclinical; Humans; Induced Pluripotent Stem Cells/drug effects/metabolism/*physiology; Neural Stem Cells/drug effects/metabolism/*physiology; Signal Transduction  
  Abstract Autism spectrum disorders (ASDs) are a heterogeneous group of neurodevelopmental disorders sharing a core set of symptoms, including impaired social interaction, language deficits, and repetitive behaviors. While ASDs are highly heritable and demonstrate a clear genetic component, the cellular and molecular mechanisms driving ASD etiology remain undefined. The unavailability of live patient-specific neurons has contributed to the difficulty in studying ASD pathophysiology. The recent advent of induced pluripotent stem cells (iPSCs) has provided the ability to generate patient-specific human neurons from somatic cells. The iPSC field has quickly grown, as researchers have demonstrated the utility of this technology to model several diseases, especially neurologic disorders. Here, we review the current literature around using iPSCs to model ASDs, and discuss the notable findings, and the promise and limitations of this technology. The recent report of a nonsyndromic ASD iPSC model and several previous ASD models demonstrating similar results points to the ability of iPSC to reveal potential novel biomarkers and therapeutics.  
  Address School of Medicine, Department of Pediatrics/Rady Children's Hospital San Diego, Department of Cellular and Molecular Medicine, Stem Cell Program, University of California San Diego, MC 0695, La Jolla, CA, 92093, USA  
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  Language English Summary Language Original Title  
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  Series Volume Series Issue Edition  
  ISSN 1878-7479 ISBN Medium  
  Area Expedition Conference  
  Notes PMID:25851569; PMCID:PMC4489954 Approved no  
  Call Number refbase @ user @ Serial 16774  
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Author (up) Agila, W.; Cajo, R.; Plaza, D. openurl 
  Title Experts agents in PEM fuel cell control Type Conference Article
  Year 2015 Publication Renewable Energy Research and Applications (ICRERA), 2015 International Conference on Abbreviated Journal  
  Volume Issue Pages 896-900  
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  Publisher Place of Publication Editor Ieee,  
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  Notes Approved no  
  Call Number refbase @ user @ agila2015experts Serial 17110  
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Author (up) Aguilera, C.A.; Sappa, A.D.; Toledo, R. openurl 
  Title Lghd: A feature descriptor for matching across non-linear intensity variations Type Conference Article
  Year 2015 Publication Image Processing (ICIP), 2015 IEEE International Conference on Abbreviated Journal  
  Volume Issue Pages 178-181  
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  Publisher Place of Publication Editor Ieee,  
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  Notes Approved no  
  Call Number refbase @ user @ aguilera2015lghd Serial 17117  
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Author (up) Andoh-Noda, T.; Akamatsu, W.; Miyake, K.; Matsumoto, T.; Yamaguchi, R.; Sanosaka, T.; Okada, Y.; Kobayashi, T.; Ohyama, M.; Nakashima, K.; Kurosawa, H.; Kubota, T.; Okano, H. url  doi
openurl 
  Title Differentiation of multipotent neural stem cells derived from Rett syndrome patients is biased toward the astrocytic lineage Type Journal Article
  Year 2015 Publication Molecular Brain Abbreviated Journal Mol Brain  
  Volume 8 Issue Pages 31  
  Keywords Astrocytes/*pathology; *Cell Differentiation; Cell Line; *Cell Lineage; Child; DNA Methylation/genetics; Fibroblasts/metabolism/pathology; Glial Fibrillary Acidic Protein/genetics; Humans; Induced Pluripotent Stem Cells/pathology; Methyl-CpG-Binding Protein 2/genetics; Mosaicism; Multipotent Stem Cells/*pathology; Mutation/genetics; Neural Stem Cells/*pathology; Protein Binding; Rett Syndrome/*pathology; Twins, Monozygotic; X Chromosome Inactivation/genetics  
  Abstract BACKGROUND: Rett syndrome (RTT) is one of the most prevalent neurodevelopmental disorders in females, caused by de novo mutations in the X-linked methyl CpG-binding protein 2 gene, MECP2. Although abnormal regulation of neuronal genes due to mutant MeCP2 is thought to induce autistic behavior and impaired development in RTT patients, precise cellular mechanisms underlying the aberrant neural progression remain unclear. RESULTS: Two sets of isogenic pairs of either wild-type or mutant MECP2-expressing human induced pluripotent stem cell (hiPSC) lines were generated from a single pair of 10-year-old RTT-monozygotic (MZ) female twins. Mutant MeCP2-expressing hiPSC lines did not express detectable MeCP2 protein during any stage of differentiation. The lack of MeCP2 reflected altered gene expression patterns in differentiated neural cells rather than in undifferentiated hiPSCs, as assessed by microarray analysis. Furthermore, MeCP2 deficiency in the neural cell lineage increased astrocyte-specific differentiation from multipotent neural stem cells. Additionally, chromatin immunoprecipitation (ChIP) and bisulfite sequencing assays indicated that anomalous glial fibrillary acidic protein gene (GFAP) expression in the MeCP2-negative, differentiated neural cells resulted from the absence of MeCP2 binding to the GFAP gene. CONCLUSIONS: An isogenic RTT-hiPSC model demonstrated that MeCP2 participates in the differentiation of neural cells. Moreover, MeCP2 deficiency triggers perturbation of astrocytic gene expression, yielding accelerated astrocyte formation from RTT-hiPSC-derived neural stem cells. These findings are likely to shed new light on astrocytic abnormalities in RTT, and suggest that astrocytes, which are required for neuronal homeostasis and function, might be a new target of RTT therapy.  
  Address Department of Physiology, Keio University School of Medicine, 35 Shinanomachi,Shinjuku-ku, Tokyo, 160-8582, Japan. hidokano@a2.keio.jp  
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  Series Volume Series Issue Edition  
  ISSN 1756-6606 ISBN Medium  
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  Notes PMID:26012557; PMCID:PMC4446051 Approved no  
  Call Number refbase @ user @ Serial 16759  
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Author (up) Ardhanareeswaran, K.; Coppola, G.; Vaccarino, F. url  openurl
  Title The use of stem cells to study autism spectrum disorder Type Journal Article
  Year 2015 Publication The Yale Journal of Biology and Medicine Abbreviated Journal Yale J Biol Med  
  Volume 88 Issue 1 Pages 5-16  
  Keywords Animals; Autism Spectrum Disorder/*pathology/therapy; Humans; Induced Pluripotent Stem Cells/*pathology; *Models, Biological; Nervous System/pathology; Stem Cell Transplantation; autism; autism spectrum disorder; induced pluripotent stem cells; stem cells  
  Abstract Autism spectrum disorder (ASD) affects as many as 1 in 68 children and is said to be the fastest-growing serious developmental disability in the United States. There is currently no medical cure or diagnostic test for ASD. Furthermore, the U.S. Food and Drug Administration has yet to approve a single drug for the treatment of autism's core symptoms. Despite numerous genome studies and the identification of hundreds of genes that may cause or predispose children to ASD, the pathways underlying the pathogenesis of idiopathic ASD still remain elusive. Post-mortem brain samples, apart from being difficult to obtain, offer little insight into a disorder that arises through the course of development. Furthermore, ASD is a disorder of highly complex, human-specific behaviors, making it difficult to model in animals. Stem cell models of ASD can be generated by performing skin biopsies of ASD patients and then dedifferentiating these fibroblasts into human-induced pluripotent stem cells (hiPSCs). iPSCs closely resemble embryonic stem cells and retain the unique genetic signature of the ASD patient from whom they were originally derived. Differentiation of these iPSCs into neurons essentially recapitulates the ASD patient's neuronal development in a dish, allowing for a patient-specific model of ASD. Here we review our current understanding of the underlying neurobiology of ASD and how the use of stem cells can advance this understanding, possibly leading to new therapeutic avenues.  
  Address Child Study Center, Yale School of Medicine, New Haven, Connecticut ; Program in Neurodevelopment and Regeneration, Yale School of Medicine, New Haven, Connecticut ; Yale Kavli Institute for Neuroscience, Yale School of Medicine, New Haven, Connecticut ; Department of Neurobiology, Yale School of Medicine, New Haven, Connecticut  
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  Series Volume Series Issue Edition  
  ISSN 0044-0086 ISBN Medium  
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  Notes PMID:25745370; PMCID:PMC4345539 Approved no  
  Call Number refbase @ user @ Serial 16780  
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