The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine
In recent years several genes have linked amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) as a spectrum disease; however little is known about what triggers their onset. With the ability to generate patient specific stem cell lines from somatic cells, it is possible to model di...
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Stem Cells International |
| Online Access: | http://dx.doi.org/10.1155/2016/9279516 |
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| author | Erin C. Hedges Vera J. Mehler Agnes L. Nishimura |
| author_facet | Erin C. Hedges Vera J. Mehler Agnes L. Nishimura |
| author_sort | Erin C. Hedges |
| collection | DOAJ |
| description | In recent years several genes have linked amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) as a spectrum disease; however little is known about what triggers their onset. With the ability to generate patient specific stem cell lines from somatic cells, it is possible to model disease without the need to transfect cells with exogenous DNA. These pluripotent stem cells have opened new avenues for identification of disease phenotypes and their relation to specific molecular pathways. Thus, as never before, compounds with potential applications for regenerative medicine can be specifically tailored in patient derived cultures. In this review, we discuss how patient specific induced pluripotent stem cells (iPSCs) have been used to model ALS and FTD and the most recent drug screening targets for these diseases. We also discuss how an iPSC bank would improve the quality of the available cell lines and how it would increase knowledge about the ALS/FTD disease spectrum. |
| format | Article |
| id | doaj-art-089417ad9bb54598bd05ce7d55ae258a |
| institution | OA Journals |
| issn | 1687-966X 1687-9678 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Stem Cells International |
| spelling | doaj-art-089417ad9bb54598bd05ce7d55ae258a2025-08-20T02:03:20ZengWileyStem Cells International1687-966X1687-96782016-01-01201610.1155/2016/92795169279516The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative MedicineErin C. Hedges0Vera J. Mehler1Agnes L. Nishimura2Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience (IOPPN), King’s College London and King’s Health Partners, London SE5 9RX, UKDepartment of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience (IOPPN), King’s College London and King’s Health Partners, London SE5 9RX, UKDepartment of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience (IOPPN), King’s College London and King’s Health Partners, London SE5 9RX, UKIn recent years several genes have linked amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) as a spectrum disease; however little is known about what triggers their onset. With the ability to generate patient specific stem cell lines from somatic cells, it is possible to model disease without the need to transfect cells with exogenous DNA. These pluripotent stem cells have opened new avenues for identification of disease phenotypes and their relation to specific molecular pathways. Thus, as never before, compounds with potential applications for regenerative medicine can be specifically tailored in patient derived cultures. In this review, we discuss how patient specific induced pluripotent stem cells (iPSCs) have been used to model ALS and FTD and the most recent drug screening targets for these diseases. We also discuss how an iPSC bank would improve the quality of the available cell lines and how it would increase knowledge about the ALS/FTD disease spectrum.http://dx.doi.org/10.1155/2016/9279516 |
| spellingShingle | Erin C. Hedges Vera J. Mehler Agnes L. Nishimura The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine Stem Cells International |
| title | The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine |
| title_full | The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine |
| title_fullStr | The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine |
| title_full_unstemmed | The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine |
| title_short | The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine |
| title_sort | use of stem cells to model amyotrophic lateral sclerosis and frontotemporal dementia from basic research to regenerative medicine |
| url | http://dx.doi.org/10.1155/2016/9279516 |
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