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  • 2005-2009  (304)
  • 2008  (304)
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  • 2005-2009  (304)
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  • 1
    Publication Date: 2008-12-23
    Description: Spinal muscular atrophy is one of the most common inherited forms of neurological disease leading to infant mortality. Patients have selective loss of lower motor neurons resulting in muscle weakness, paralysis and often death. Although patient fibroblasts have been used extensively to study spinal muscular atrophy, motor neurons have a unique anatomy and physiology which may underlie their vulnerability to the disease process. Here we report the generation of induced pluripotent stem cells from skin fibroblast samples taken from a child with spinal muscular atrophy. These cells expanded robustly in culture, maintained the disease genotype and generated motor neurons that showed selective deficits compared to those derived from the child's unaffected mother. This is the first study to show that human induced pluripotent stem cells can be used to model the specific pathology seen in a genetically inherited disease. As such, it represents a promising resource to study disease mechanisms, screen new drug compounds and develop new therapies.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659408/" 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/PMC2659408/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Ebert, Allison D -- Yu, Junying -- Rose, Ferrill F Jr -- Mattis, Virginia B -- Lorson, Christian L -- Thomson, James A -- Svendsen, Clive N -- P01 NS057778/NS/NINDS NIH HHS/ -- P01 NS057778-01A1/NS/NINDS NIH HHS/ -- P01 NS057778-01A10002/NS/NINDS NIH HHS/ -- P01 NS057778-01A18867/NS/NINDS NIH HHS/ -- P01 NS057778-01A19001/NS/NINDS NIH HHS/ -- P01 NS057778-02/NS/NINDS NIH HHS/ -- P01 NS057778-020002/NS/NINDS NIH HHS/ -- P01 NS057778-029001/NS/NINDS NIH HHS/ -- P01NS057778/NS/NINDS NIH HHS/ -- R01HD054413/HD/NICHD NIH HHS/ -- R01NS41584/NS/NINDS NIH HHS/ -- T32 AG027566/AG/NIA NIH HHS/ -- T32 AG027566-01/AG/NIA NIH HHS/ -- T32 AG027566-02/AG/NIA NIH HHS/ -- T32 AG027566-03/AG/NIA NIH HHS/ -- T32 AG027566-04/AG/NIA NIH HHS/ -- T32GM008396/GM/NIGMS NIH HHS/ -- England -- Nature. 2009 Jan 15;457(7227):277-80. doi: 10.1038/nature07677. Epub 2008 Dec 21.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉The Waisman Center, The Stem Cell and Regenerative Medicine Center, University of Wisconsin-Madison, 1500 Highland Avenue, Madison, Wisconsin 53705, USA. ebert@waisman.wisc.edu〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19098894" target="_blank"〉PubMed〈/a〉
    Keywords: Cell Differentiation/drug effects ; Cell Lineage ; Cell Separation ; Cells, Cultured ; *Cellular Reprogramming/drug effects ; Child ; Female ; Fibroblasts/*cytology/drug effects ; Gene Expression Regulation/drug effects ; Humans ; Male ; *Models, Biological ; Motor Neurons/drug effects/metabolism/*pathology ; Muscular Atrophy, Spinal/metabolism/*pathology ; Pluripotent Stem Cells/*cytology/drug effects/metabolism/*pathology ; Skin/cytology ; Survival of Motor Neuron 1 Protein/genetics/metabolism
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 2
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    American Association for the Advancement of Science (AAAS)
    Publication Date: 2008-12-20
    Description: Nuclear reprogramming describes a switch in gene expression of one kind of cell to that of another unrelated cell type. Early studies in frog cloning provided some of the first experimental evidence for reprogramming. Subsequent procedures included mammalian somatic cell nuclear transfer, cell fusion, induction of pluripotency by ectopic gene expression, and direct reprogramming. Through these methods it becomes possible to derive one kind of specialized cell (such as a brain cell) from another, more accessible, tissue (such as skin) in the same individual. This has potential applications for cell replacement without the immunosuppression treatments that are required when cells are transferred between genetically different individuals. This article provides some background to this field, a discussion of mechanisms and efficiency, and comments on prospects for future nuclear reprogramming research.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Gurdon, J B -- Melton, D A -- Wellcome Trust/United Kingdom -- New York, N.Y. -- Science. 2008 Dec 19;322(5909):1811-5. doi: 10.1126/science.1160810.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Wellcome Trust/Cancer Research UK Gurdon Institute and Department of Zoology, University of Cambridge, Cambridge CB2 12N, UK.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19095934" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Cell Dedifferentiation ; Cell Differentiation ; Cell Fusion ; Cell Lineage ; *Cellular Reprogramming ; Cloning, Organism ; DNA/metabolism ; DNA-Binding Proteins/metabolism ; Embryonic Stem Cells/cytology/physiology ; Female ; Gene Expression ; Humans ; Male ; Nuclear Transfer Techniques ; Oocytes/cytology ; Pluripotent Stem Cells/cytology/physiology ; Regulatory Sequences, Nucleic Acid ; Transcription Factors/genetics/metabolism
    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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  • 3
    Publication Date: 2008-12-20
    Description: The repeated discovery of adult dinosaurs in close association with egg clutches leads to speculation over the type and extent of care exhibited by these extinct animals for their eggs and young. To assess parental care in Cretaceous troodontid and oviraptorid dinosaurs, we examined clutch volume and the bone histology of brooding adults. In comparison to four archosaur care regressions, the relatively large clutch volumes of Troodon, Oviraptor, and Citipati scale most closely with a bird-paternal care model. Clutch-associated adults lack the maternal and reproductively associated histologic features common to extant archosaurs. Large clutch volumes and a suite of reproductive features shared only with birds favor paternal care, possibly within a polygamous mating system. Paternal care in both troodontids and oviraptorids indicates that this care system evolved before the emergence of birds and represents birds' ancestral condition. In extant birds and over most adult sizes, paternal and biparental care correspond to the largest and smallest relative clutch volumes, respectively.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Varricchio, David J -- Moore, Jason R -- Erickson, Gregory M -- Norell, Mark A -- Jackson, Frankie D -- Borkowski, John J -- New York, N.Y. -- Science. 2008 Dec 19;322(5909):1826-8. doi: 10.1126/science.1163245.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Earth Sciences, Montana State University, Bozeman, MT 59717, USA. djv@montana.edu〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19095938" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; *Behavior, Animal ; *Biological Evolution ; *Birds/physiology ; Bone and Bones/anatomy & histology ; Clutch Size ; *Dinosaurs/physiology ; Female ; *Fossils ; Male ; Maternal Behavior ; *Nesting Behavior ; Paternal Behavior ; Regression Analysis ; *Sexual Behavior, Animal
    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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  • 4
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    American Association for the Advancement of Science (AAAS)
    Publication Date: 2008-12-20
    Description: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Prum, Richard O -- New York, N.Y. -- Science. 2008 Dec 19;322(5909):1799-800. doi: 10.1126/science.1168808.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Ecology and Evolutionary Biology and Peabody Museum of Natural History, Post Office Box 208105, Yale University, New Haven, CT 06520, USA. richard.prum@yale.edu〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19095929" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; *Behavior, Animal ; *Biological Evolution ; *Birds/physiology ; Clutch Size ; *Dinosaurs/physiology ; Female ; *Fossils ; Male ; *Nesting Behavior ; Paternal Behavior ; Sexual Behavior, Animal
    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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  • 5
    Publication Date: 2008-12-20
    Description: We report the existence of an entorhinal cell type that fires when an animal is close to the borders of the proximal environment. The orientation-specific edge-apposing activity of these "border cells" is maintained when the environment is stretched and during testing in enclosures of different size and shape in different rooms. Border cells are relatively sparse, making up less than 10% of the local cell population, but can be found in all layers of the medial entorhinal cortex as well as the adjacent parasubiculum, often intermingled with head-direction cells and grid cells. Border cells may be instrumental in planning trajectories and anchoring grid fields and place fields to a geometric reference frame.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Solstad, Trygve -- Boccara, Charlotte N -- Kropff, Emilio -- Moser, May-Britt -- Moser, Edvard I -- New York, N.Y. -- Science. 2008 Dec 19;322(5909):1865-8. doi: 10.1126/science.1166466.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Kavli Institute for Systems Neuroscience and Centre for the Biology of Memory, Norwegian University of Science and Technology, 7489 Trondheim, Norway.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19095945" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Brain Mapping ; Cues ; Electrophysiology ; Entorhinal Cortex/*cytology/*physiology ; Male ; Neurons/*physiology ; *Orientation ; Rats ; Rats, Long-Evans ; *Space Perception
    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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  • 6
    Publication Date: 2008-12-20
    Description: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Koenig, Robert -- New York, N.Y. -- Science. 2008 Dec 19;322(5909):1778. doi: 10.1126/science.322.5909.1778.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19095913" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Conservation of Natural Resources ; Democratic Republic of the Congo ; Ecosystem ; Female ; *Gorilla gorilla/genetics ; Habituation, Psychophysiologic ; Male ; Population Density ; Violence
    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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  • 7
    Publication Date: 2008-12-17
    Description: Behavioural studies widely implicate sleep in memory consolidation in the learning of a broad range of behaviours. During sleep, brain regions are reactivated, and specific patterns of neural activity are replayed, consistent with patterns observed in previous waking behaviour. Birdsong learning is a paradigmatic model system for skill learning. Song development in juvenile zebra finches (Taeniopygia guttata) is characterized by sleep-dependent circadian fluctuations in singing behaviour, with immediate post-sleep deterioration in song structure followed by recovery later in the day. In sleeping adult birds, spontaneous bursting activity of forebrain premotor neurons in the robust nucleus of the arcopallium (RA) carries information about daytime singing. Here we show that, in juvenile zebra finches, playback during the day of an adult 'tutor' song induced profound and tutor-song-specific changes in bursting activity of RA neurons during the following night of sleep. The night-time neuronal changes preceded tutor-song-induced changes in singing, first observed the following day. Interruption of auditory feedback greatly reduced sleep bursting and prevented the tutor-song-specific neuronal remodelling. Thus, night-time neuronal activity is shaped by the interaction of the song model (sensory template) and auditory feedback, with changes in night-time activity preceding the onset of practice associated with vocal learning. We hypothesize that night-time bursting induces adaptive changes in premotor networks during sleep as part of vocal learning. By this hypothesis, adaptive changes driven by replay of sensory information at night and by evaluation of sensory feedback during the day interact to produce the complex circadian patterns seen early in vocal development.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2651989/" 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/PMC2651989/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Shank, Sylvan S -- Margoliash, Daniel -- R01 MH059831/MH/NIMH NIH HHS/ -- R01 MH059831-09/MH/NIMH NIH HHS/ -- England -- Nature. 2009 Mar 5;458(7234):73-7. doi: 10.1038/nature07615. Epub 2008 Dec 14.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Psychology, University of Chicago, Chicago, Illinois 60637, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19079238" target="_blank"〉PubMed〈/a〉
    Keywords: Acoustic Stimulation ; Action Potentials ; Animals ; Circadian Rhythm/physiology ; Darkness ; Entropy ; Feedback, Physiological ; Female ; Finches/*physiology ; Learning/*physiology ; Male ; Mental Recall/physiology ; *Models, Neurological ; Neurons/physiology ; Prosencephalon/cytology/*physiology ; Sleep/*physiology ; Vocalization, Animal/*physiology
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 8
    Publication Date: 2008-12-17
    Description: Retinoic acid (RA) is an essential extrinsic inducer of meiotic initiation in mammalian germ cells. However, RA acts too widely in mammalian development to account, by itself, for the cell-type and temporal specificity of meiotic initiation. We considered parallels to yeast, in which extrinsic and intrinsic factors combine to restrict meiotic initiation. We demonstrate that, in mouse embryos, extrinsic and intrinsic factors together regulate meiotic initiation. The mouse RNA-binding protein DAZL, which is expressed by postmigratory germ cells, is a key intrinsic factor, enabling those cells to initiate meiosis in response to RA. Within a brief developmental window, Dazl-expressing germ cells in both XX and XY embryos actively acquire the ability to interpret RA as a meiosis-inducing signal.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Lin, Yanfeng -- Gill, Mark E -- Koubova, Jana -- Page, David C -- Howard Hughes Medical Institute/ -- New York, N.Y. -- Science. 2008 Dec 12;322(5908):1685-7. doi: 10.1126/science.1166340.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Howard Hughes Medical Institute, Whitehead Institute, and Department of Biology, Massachusetts Institute of Technology, 9 Cambridge Center, Cambridge, MA 02142, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19074348" target="_blank"〉PubMed〈/a〉
    Keywords: Adaptor Proteins, Signal Transducing ; Animals ; Cell Cycle Proteins/metabolism ; Cell Nucleus/ultrastructure ; DNA Breaks ; DNA Repair ; Embryo, Mammalian/*cytology/physiology ; Endodeoxyribonucleases ; Esterases/metabolism ; Female ; Germ Cells/*cytology ; Male ; *Meiosis ; Mice ; Mice, Inbred C57BL ; Nuclear Proteins/genetics/metabolism ; Ovary/embryology/physiology ; Phosphoproteins/genetics/metabolism ; Proteins/metabolism ; RNA-Binding Proteins/genetics/*physiology ; Testis/embryology/physiology ; Tretinoin/pharmacology
    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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  • 9
    Publication Date: 2008-12-17
    Description: Helgason et al. (Reports, 8 February 2008, p. 813) reported a positive association between kinship and fertility in the Icelandic population. We point out that the data further suggest that fertility initially increases with kinship and then decays. This is supported by another large study on the Danish population suggesting a superposition of effects of inbreeding and outbreeding depression on human fertility.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Labouriau, Rodrigo -- Amorim, Antonio -- New York, N.Y. -- Science. 2008 Dec 12;322(5908):1634; author reply 1634. doi: 10.1126/science.1161907.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Genetics and Biotechnology, Faculty of Agricultural Sciences, Aarhus University, DK-8830 Tjele, Denmark. rodrigo.labouriau@agrsci.dk〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19074330" target="_blank"〉PubMed〈/a〉
    Keywords: Consanguinity ; Denmark ; *Family ; *Family Characteristics ; Female ; *Fertility ; Humans ; Iceland ; Male ; Socioeconomic Factors
    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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  • 10
    Publication Date: 2008-12-17
    Description: Models of cognitive control posit a key modulatory role for the pontine locus coeruleus-norepinephrine (LC-NE) system. In nonhuman primates, phasic LC-NE activity confers adaptive adjustments in cortical gain in task-relevant brain networks, and in performance, on a trial-by-trial basis. This model has remained untested in humans. We used the pharmacological agent modafinil to promote low-tonic/high-phasic LC-NE activity in healthy humans performing a cognitive control task during event-related functional magnetic resonance imaging (fMRI). Modafanil administration was associated with decreased task-independent, tonic LC activity, increased task-related LC and prefrontal cortex (PFC) activity, and enhanced LC-PFC functional connectivity. These results confirm in humans the role of the LC-NE system in PFC function and cognitive control and suggest a mechanism for therapeutic action of procognitive noradrenergic agents.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Minzenberg, Michael J -- Watrous, Andrew J -- Yoon, Jong H -- Ursu, Stefan -- Carter, Cameron S -- MH059883/MH/NIMH NIH HHS/ -- UL1 RR024146/RR/NCRR NIH HHS/ -- New York, N.Y. -- Science. 2008 Dec 12;322(5908):1700-2. doi: 10.1126/science.1164908.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Psychiatry, University of California, Davis School of Medicine, Sacramento, CA, USA. michael.minzenberg@ucdmc.ucdavis.edu〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/19074351" target="_blank"〉PubMed〈/a〉
    Keywords: Adult ; Benzhydryl Compounds/administration & dosage/*pharmacology ; Brain Mapping ; Central Nervous System Stimulants/pharmacology ; *Cognition/drug effects ; Female ; Humans ; Locus Coeruleus/drug effects/*physiology ; Magnetic Resonance Imaging ; Male ; Neurons/drug effects/physiology ; Norepinephrine/*metabolism ; Norepinephrine Plasma Membrane Transport Proteins/antagonists & ; inhibitors/metabolism ; Prefrontal Cortex/physiology ; Task Performance and Analysis
    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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