Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells

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...

Full description

Bibliographic Details
Main Authors:	Wenting Guo, Laura Fumagalli, Robert Prior, Ludo Van Den Bosch
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2017-12-01
Series:	Frontiers in Neuroscience
Subjects:	amyotrophic lateral sclerosis frontotemporal dementia iPSC neurodegeneration motor neuron
Online Access:	http://journal.frontiersin.org/article/10.3389/fnins.2017.00671/full

Similar Items

Moderate intrinsic phenotypic alterations in C9orf72 ALS/FTD iPSC-microglia despite the presence of C9orf72 pathological features
by: Ileana Lorenzini, et al.
Published: (2023-06-01)

Translation of dipeptide repeat proteins in C9ORF72 ALS/FTD through unique and redundant AUG initiation codons
by: Yoshifumi Sonobe, et al.
Published: (2023-09-01)

A toxic gain-of-function mechanism in C9orf72 ALS impairs the autophagy-lysosome pathway in neurons
by: Jimmy Beckers, et al.
Published: (2023-09-01)

A pathogenic mutation in the ALS/FTD gene VCP induces mitochondrial hypermetabolism by modulating the permeability transition pore
by: Silke Vanderhaeghe, et al.
Published: (2024-10-01)

Distinct Phospho-TDP-43 brain distribution in two cases of FTD, one associated with ALS
by: Álvaro C.B. Guedes, et al.

Emerging perspectives of synaptic biomarkers in ALS and FTD
by: Karrthik Krishnamurthy, et al.
Published: (2024-01-01)

Neural circuit and synaptic dysfunctions in ALS-FTD pathology
by: Santiago Mora, et al.
Published: (2023-07-01)

Thalamic and Cerebellar Regional Involvement across the ALS–FTD Spectrum and the Effect of <i>C9orf72</i>
by: Martina Bocchetta, et al.
Published: (2022-03-01)

Specific vulnerability of iPSC-derived motor neurons with TDP-43 gene mutation to oxidative stress
by: Asako Onda-Ohto, et al.
Published: (2023-07-01)

The Need for Biomarkers in the ALS–FTD Spectrum: A Clinical Point of View on the Role of Proteomics
by: Francesca Vignaroli, et al.
Published: (2023-01-01)

The era of cryptic exons: implications for ALS-FTD
by: Puja R. Mehta, et al.
Published: (2023-03-01)

Editorial: The role of glial cells in neurodegeneration
by: Piera Pasinelli, et al.
Published: (2024-01-01)

Exome Sequencing of a Portuguese Cohort of Frontotemporal Dementia Patients: Looking Into the ALS-FTD Continuum
by: Miguel Tábuas-Pereira, et al.
Published: (2022-07-01)

Proteomics Approaches for Biomarker and Drug Target Discovery in ALS and FTD
by: Thomas J. Hedl, et al.
Published: (2019-06-01)

Cellular Stress Induces Nucleocytoplasmic Transport Deficits Independent of Stress Granules
by: Joni Vanneste, et al.
Published: (2022-05-01)

The association between lifelong personality and clinical phenotype in the FTD-ALS spectrum
by: Giulia Vinceti, et al.
Published: (2023-10-01)

TDP-43 Proteinopathy Specific Biomarker Development
by: Isabell Cordts, et al.
Published: (2023-02-01)

Editorial: What's next? Innovative translational markers across the ALS-FTD continuum
by: Fabiola De Marchi, et al.
Published: (2023-08-01)

Cognitive and Behavioral Manifestations in ALS: Beyond Motor System Involvement
by: Robert Rusina, et al.
Published: (2021-03-01)

Linking hnRNP Function to ALS and FTD Pathology
by: Maria D. Purice, et al.
Published: (2018-05-01)

C9orf72 gene networks in the human brain correlate with cortical thickness in C9-FTD and implicate vulnerable cell types
by: Iris J. Broce, et al.
Published: (2024-02-01)

Frontotemporal dementia and amyotrophic lateral sclerosis: Revisiting one of the first case reports with neuropathological examination
by: Ricardo Nitrini

Clinical and neuropathological features of ALS/FTD with TIA1 mutations
by: Veronica Hirsch-Reinshagen, et al.
Published: (2017-12-01)

Editorial: Mechanisms of Action in Neurodegenerative Proteinopathies
by: Sean J. Miller, et al.
Published: (2022-06-01)

The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
by: Yevgeniya A. Abramzon, et al.
Published: (2020-02-01)

Organ on a Chip: A Novel in vitro Biomimetic Strategy in Amyotrophic Lateral Sclerosis (ALS) Modeling
by: Babak Arjmand, et al.
Published: (2022-01-01)

DNA Damage and Repair Deficiency in ALS/FTD-Associated Neurodegeneration: From Molecular Mechanisms to Therapeutic Implication
by: Haibo Wang, et al.
Published: (2021-12-01)

Nuclear-import receptors as gatekeepers of pathological phase transitions in ALS/FTD
by: Bilal Khalil, et al.
Published: (2024-01-01)

Telomere Attrition in Induced Pluripotent Stem Cell-Derived Neurons From ALS/FTD-Related C9ORF72 Repeat Expansion Carriers
by: Hayley Robinson, et al.
Published: (2022-06-01)

G‐quadruplex‐binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo
by: Roberto Simone, et al.
Published: (2017-11-01)

Immunotherapy targeting the C-terminal domain of TDP-43 decreases neuropathology and confers neuroprotection in mouse models of ALS/FTD
by: Tariq Afroz, et al.
Published: (2023-04-01)

Increased Risk of Aging-Related Neurodegenerative Disease after Traumatic Brain Injury
by: Sarah Barker, et al.
Published: (2023-04-01)

Alzheimer’s in a dish – induced pluripotent stem cell-based disease modeling
by: Sherida de Leeuw, et al.
Published: (2019-07-01)

β-N-Methylamino-L-Alanine Induces Neurological Deficits and Shortened Life Span in Drosophila
by: R. Grace Zhai, et al.
Published: (2010-11-01)

Emerging Trends in the Field of Inflammation and Proteinopathy in ALS/FTD Spectrum Disorder
by: Fabiola De Marchi, et al.
Published: (2023-05-01)

Impairments of inhibitory neurons in amyotrophic lateral sclerosis and frontotemporal dementia
by: Félicie Lorenc, et al.
Published: (2024-12-01)

Motor neuron disease and frontotemporal dementia: One, two, or three diseases?
by: Bak Thomas
Published: (2010-10-01)

Active poly‐GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model
by: Qihui Zhou, et al.
Published: (2019-12-01)

Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo
by: Henrick Riemenschneider, et al.
Published: (2023-07-01)

Deregulated expression of a longevity gene, Klotho, in the C9orf72 deletion mice with impaired synaptic plasticity and adult hippocampal neurogenesis
by: Ho, Wan Yun, et al.
Published: (2021)