Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two age-dependent multifactorial neurodegenerative disorders, which are typically characterized by the selective death of motor neurons and cerebral cortex neurons, respectively. These two diseases share many clinical, genetic...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2017-12-01
|
| Series: | Frontiers in Neuroscience |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/article/10.3389/fnins.2017.00671/full |
| _version_ | 1818216535173365760 |
|---|---|
| author | Wenting Guo Wenting Guo Laura Fumagalli Laura Fumagalli Robert Prior Robert Prior Ludo Van Den Bosch Ludo Van Den Bosch |
| author_facet | Wenting Guo Wenting Guo Laura Fumagalli Laura Fumagalli Robert Prior Robert Prior Ludo Van Den Bosch Ludo Van Den Bosch |
| author_sort | Wenting Guo |
| collection | DOAJ |
| description | Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two age-dependent multifactorial neurodegenerative disorders, which are typically characterized by the selective death of motor neurons and cerebral cortex neurons, respectively. These two diseases share many clinical, genetic and pathological aspects. During the past decade, cell reprogramming technologies enabled researchers to generate human induced pluripotent stem cells (iPSCs) from somatic cells. This resulted in the unique opportunity to obtain specific neuronal and non-neuronal cell types from patients which could be used for basic research. Moreover, these in vitro models can mimic not only the familial forms of ALS/FTD, but also sporadic cases without known genetic cause. At present, there have been extensive technical advances in the generation of iPSCs, as well as in the differentiation procedures to obtain iPSC-derived motor neurons, cortical neurons and non-neuronal cells. The major challenge at this moment is to determine whether these iPSC-derived cells show relevant phenotypes that recapitulate complex diseases. In this review, we will summarize the work related to iPSC models of ALS and FTD. In addition, we will discuss potential drawbacks and solutions for establishing more trustworthy iPSC models for both ALS and FTD. |
| first_indexed | 2024-12-12T06:53:31Z |
| format | Article |
| id | doaj.art-647397866aa74e128ae57d8e53a63397 |
| institution | Directory Open Access Journal |
| issn | 1662-453X |
| language | English |
| last_indexed | 2024-12-12T06:53:31Z |
| publishDate | 2017-12-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neuroscience |
| spelling | doaj.art-647397866aa74e128ae57d8e53a633972022-12-22T00:34:00ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2017-12-011110.3389/fnins.2017.00671308102Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem CellsWenting Guo0Wenting Guo1Laura Fumagalli2Laura Fumagalli3Robert Prior4Robert Prior5Ludo Van Den Bosch6Ludo Van Den Bosch7KU Leuven-Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease, Leuven, BelgiumLaboratory of Neurobiology, VIB & KU Leuven Center for Brain & Disease Research, Leuven, BelgiumKU Leuven-Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease, Leuven, BelgiumLaboratory of Neurobiology, VIB & KU Leuven Center for Brain & Disease Research, Leuven, BelgiumKU Leuven-Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease, Leuven, BelgiumLaboratory of Neurobiology, VIB & KU Leuven Center for Brain & Disease Research, Leuven, BelgiumKU Leuven-Department of Neurosciences, Experimental Neurology and Leuven Institute for Neuroscience and Disease, Leuven, BelgiumLaboratory of Neurobiology, VIB & KU Leuven Center for Brain & Disease Research, Leuven, BelgiumAmyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two age-dependent multifactorial neurodegenerative disorders, which are typically characterized by the selective death of motor neurons and cerebral cortex neurons, respectively. These two diseases share many clinical, genetic and pathological aspects. During the past decade, cell reprogramming technologies enabled researchers to generate human induced pluripotent stem cells (iPSCs) from somatic cells. This resulted in the unique opportunity to obtain specific neuronal and non-neuronal cell types from patients which could be used for basic research. Moreover, these in vitro models can mimic not only the familial forms of ALS/FTD, but also sporadic cases without known genetic cause. At present, there have been extensive technical advances in the generation of iPSCs, as well as in the differentiation procedures to obtain iPSC-derived motor neurons, cortical neurons and non-neuronal cells. The major challenge at this moment is to determine whether these iPSC-derived cells show relevant phenotypes that recapitulate complex diseases. In this review, we will summarize the work related to iPSC models of ALS and FTD. In addition, we will discuss potential drawbacks and solutions for establishing more trustworthy iPSC models for both ALS and FTD.http://journal.frontiersin.org/article/10.3389/fnins.2017.00671/fullamyotrophic lateral sclerosisfrontotemporal dementiaiPSCneurodegenerationmotor neuron |
| spellingShingle | Wenting Guo Wenting Guo Laura Fumagalli Laura Fumagalli Robert Prior Robert Prior Ludo Van Den Bosch Ludo Van Den Bosch Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells Frontiers in Neuroscience amyotrophic lateral sclerosis frontotemporal dementia iPSC neurodegeneration motor neuron |
| title | Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells |
| title_full | Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells |
| title_fullStr | Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells |
| title_full_unstemmed | Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells |
| title_short | Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells |
| title_sort | current advances and limitations in modeling als ftd in a dish using induced pluripotent stem cells |
| topic | amyotrophic lateral sclerosis frontotemporal dementia iPSC neurodegeneration motor neuron |
| url | http://journal.frontiersin.org/article/10.3389/fnins.2017.00671/full |
| work_keys_str_mv | AT wentingguo currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT wentingguo currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT laurafumagalli currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT laurafumagalli currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT robertprior currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT robertprior currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT ludovandenbosch currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells AT ludovandenbosch currentadvancesandlimitationsinmodelingalsftdinadishusinginducedpluripotentstemcells |