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557 results on '"WILMUT, IAN"'

2. Comment on “Drug Screening for ALS Using Patient-Specific Induced Pluripotent Stem Cells”

Author

Bilican, Bilada, Serio, Andrea, Barmada, Sami J, Nishimura, Agnes Lumi, Sullivan, Gareth J, Carrasco, Monica, Phatnani, Hemali P, Puddifoot, Clare A, Story, David, Fletcher, Judy, Park, In-Hyun, Friedman, Brad A, Daley, George Q, Wyllie, David JA, Hardingham, Giles E, Wilmut, Ian, Finkbeiner, Steven, Maniatis, Tom, Shaw, Christopher E, and Chandran, Siddharthan

Subjects
Medical Biotechnology, Biomedical and Clinical Sciences, Neurodegenerative, Stem Cell Research - Induced Pluripotent Stem Cell - Human, Stem Cell Research, Stem Cell Research - Embryonic - Human, Stem Cell Research - Induced Pluripotent Stem Cell - Non-Human, Neurosciences, ALS, Clinical Research, Rare Diseases, Stem Cell Research - Induced Pluripotent Stem Cell, Regenerative Medicine, Brain Disorders, Aetiology, 2.1 Biological and endogenous factors, Neurological, Amyotrophic Lateral Sclerosis, Drug Evaluation, Preclinical, Humans, Induced Pluripotent Stem Cells, Motor Neurons, Biological Sciences, Medical and Health Sciences, Medical biotechnology, Biomedical engineering
Abstract

Egawa et al. recently showed the value of patient-specific induced pluripotent stem cells (iPSCs) for modeling amyotrophic lateral sclerosis in vitro. Their study and our work highlight the need for complementary assays to detect small, but potentially important, phenotypic differences between control iPSC lines and those carrying disease mutations.

Published
2013

3. Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy

Author

Serio, Andrea, Bilican, Bilada, Barmada, Sami J, Ando, Dale Michael, Zhao, Chen, Siller, Rick, Burr, Karen, Haghi, Ghazal, Story, David, Nishimura, Agnes Lumi, Carrasco, Monica A, Phatnani, Hemali P, Shum, Carole, Wilmut, Ian, Maniatis, Tom, Shaw, Christopher E, Finkbeiner, Steven, and Chandran, Siddharthan

Subjects
Biochemistry and Cell Biology, Biomedical and Clinical Sciences, Biological Sciences, Rare Diseases, Stem Cell Research - Induced Pluripotent Stem Cell, Clinical Research, Stem Cell Research - Nonembryonic - Human, Stem Cell Research, Neurosciences, Brain Disorders, ALS, Neurodegenerative, 2.1 Biological and endogenous factors, Aetiology, Neurological, Amyotrophic Lateral Sclerosis, Astrocytes, Cell Line, Cell Proliferation, Cell Survival, Coculture Techniques, DNA-Binding Proteins, Humans, Induced Pluripotent Stem Cells, Male, Middle Aged, Motor Neurons, Mutation, glia, motor neuron disease, disease modeling
Abstract

Glial proliferation and activation are associated with disease progression in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia. In this study, we describe a unique platform to address the question of cell autonomy in transactive response DNA-binding protein (TDP-43) proteinopathies. We generated functional astroglia from human induced pluripotent stem cells carrying an ALS-causing TDP-43 mutation and show that mutant astrocytes exhibit increased levels of TDP-43, subcellular mislocalization of TDP-43, and decreased cell survival. We then performed coculture experiments to evaluate the effects of M337V astrocytes on the survival of wild-type and M337V TDP-43 motor neurons, showing that mutant TDP-43 astrocytes do not adversely affect survival of cocultured neurons. These observations reveal a significant and previously unrecognized glial cell-autonomous pathological phenotype associated with a pathogenic mutation in TDP-43 and show that TDP-43 proteinopathies do not display an astrocyte non-cell-autonomous component in cell culture, as previously described for SOD1 ALS. This study highlights the utility of induced pluripotent stem cell-based in vitro disease models to investigate mechanisms of disease in ALS and other TDP-43 proteinopathies.

Published
2013

4. The Future of STEM CELLS

Author

Barber, Lionel, Rennie, John, Cookson, Clive, Soares, Christine, Gardner, Richard, Watson, Tim, Waldmeir, Patti, Stix, Gary, Beardsley, Sara, Gibbs, W. Wayt, Weissman, Irving, Burton, John, Griffith, Victoria, Moran, Nuala, and Wilmut, Ian

Published
2005

14. Dolly Confirmation

Author

Sgaramella, Vittorio, Zinder, Norton D., Colman, Alan, and Wilmut, Ian

Published
1998

17. Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability

Author

Bilican, Bilada, Serio, Andrea, Barmada, Sami J., Nishimura, Agnes Lumi, Sullivan, Gareth J., Carrasco, Monica, Phatnani, Hemali P., Puddifoot, Clare A., Story, David, Fletcher, Judy, Park, In-Hyun, Friedman, Brad A., Daley, George Q., Wyllie, David J. A., Hardingham, Giles E., Wilmut, Ian, Finkbeiner, Steven, Maniatis, Tom, Shaw, Christopher E., and Chandran, Siddharthan

Published
2012

22. The ISSCR Guidelines for Human Embryonic Stem Cell Research

Author

Daley, George O., Ahrlund-Richter, Lars, Auerbach, Jonathan M., Benvenisty, Nissim, Charo, R. Alta, Chen, Grace, Deng, Hong-kui, Goldstein, Lawrence S., Hudson, Kathy L., Hyun, Insoo, Junn, Sung Chull, Love, Jane, Lee, Eng Hin, McLaren, Anne, Mummery, Christine L., Nakatsuji, Norio, Racowsky, Catherine, Rooke, Heather, Rossant, Janet, Schöler, Hans R., Solbakk, Jan Helge, Taylor, Patrick, Trounson, Alan O., Weissman, Irving L., Wilmut, Ian, Yu, John, and Zoloth, Laurie

Published
2007
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30. Preface

Author

Mummery, Christine, primary, Roelen, Bernard, additional, Stolpe, Anja van de, additional, and Wilmut, Ian, additional

Published
2011
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35. Somatic Cell Nuclear Transfer

Author

Sousa, Paul De, primary, Ritchie, William, additional, Dinnyes, Andras, additional, King, Tim, additional, Paterson, Lesley, additional, and Wilmut, Ian, additional

Published
2004
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39. CONTRIBUTORS

Author

Anagnostopoulos, Anna V., primary, Attal, J., additional, Burnside, Amy S., additional, Chan, A.W.S., additional, Chong, K.Y., additional, Davisson, Muriel T., additional, Doetschman, Thomas, additional, Döpke, H.H., additional, Galat, Vasiliy, additional, Godke, Robert A., additional, Hadeler, K.G., additional, Houdebine, L.M., additional, Iannaccone, Philip, additional, Irwin, M.H., additional, Jay, Gilbert, additional, Kato, Yoko, additional, Kim, Teoan, additional, Martin, M.J., additional, Mobraaten, Larry E., additional, Mozdziak, P.E., additional, Ngo, Lien, additional, Niemann, H., additional, Overbeek, Paul A., additional, Paterson, Lesley, additional, Petitte, J.N., additional, Piedrahita, Jorge A., additional, Pinkert, Carl A., additional, Polites, H.G., additional, Pogozelski, W.K., additional, Ritchie, William, additional, Robl, James M., additional, Rucker, Edmund B., additional, Sansinena, Marina, additional, Schatten, G., additional, Thomson, James G., additional, Tinkle, Brad T., additional, Tsunoda, Yukio, additional, Vilotte, J.L., additional, Wilmut, Ian, additional, Winn, Richard N., additional, and Youngs, Curtis R., additional

Published
2002
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46. Sodium channel current loss of function in induced pluripotent stem cell-derived cardiomyocytes from a Brugada syndrome patient

Author

Selga Coma, Elisabet, Sendfeld, Franziska, Martínez Moreno, Rebecca, Medine, Claire N., Tura Ceide, Olga, Wilmut, Ian, Pérez González, Guillermo J., Scornik, Fabiana S., Brugada, Ramon, and Mills, Nicholas L.

Subjects
Electrophysiology, Brugada, Síndrome de, Journal Article, Cardiology, Brugada, Síndrome, Brugada syndrome, Stem cells, Electrofisiologia, Cèl·lules mare, Cardiologia
Abstract

Brugada syndrome predisposes to sudden death due to disruption of normal cardiac ion channel function, yet our understanding of the underlying cellular mechanisms is incomplete. Commonly used heterologous expression models lack many characteristics of native cardiomyocytes and, in particular, the individual genetic background of a patient. Patient-specific induced pluripotent stem (iPS) cell-derived cardiomyocytes (iPS-CM) may uncover cellular phenotypical characteristics not observed in heterologous models. Our objective was to determine the properties of the sodium current in iPS-CM with a mutation in SCN5A associated with Brugada syndrome. Dermal fibroblasts from a Brugada syndrome patient with a mutation in SCN5A (c.1100G>A, leading to Nav1.5_p.R367H) were reprogrammed to iPS cells. Clones were characterized and differentiated to form beating clusters and sheets. Patient and control iPS-CM were structurally indistinguishable. Sodium current properties of patient and control iPS-CM were compared. These results were contrasted with those obtained in tsA201 cells heterologously expressing sodium channels with the same mutation. Patient-derived iPS-CM showed a 33.1-45.5% reduction in INa density, a shift in both activation and inactivation voltage-dependence curves, and faster recovery from inactivation. Co-expression of wild-type and mutant channels in tsA201 cells did not compromise channel trafficking to the membrane, but resulted in a reduction of 49.8% in sodium current density without affecting any other parameters. Cardiomyocytes derived from iPS cells from a Brugada syndrome patient with a mutation in SCN5A recapitulate the loss of function of sodium channel current associated with this syndrome; including pro-arrhythmic changes in channel function not detected using conventional heterologous expression systems.

Published
2018

47. DNA microsatellite analysis of Dolly

Author

Ashworth, David, Bishop, Matthew, Campbell, Keith, Colman, Alan, Kind, Alex, Schnieke, Angelika, Blott, Sarah, Griffin, Harry, Haley, Chris, McWhir, Jim, and Wilmut, Ian

Published
1998

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