In Vivo and In Vitro Models to Study Amyotrophic Lateral Sclerosis

Berthod, François; Gros‐Louis, François

doi:10.5772/39076

Cited by 8 publications

(8 citation statements)

References 185 publications

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“…For this reason, and for the complex multi-cellular system present in ALS, 2-D cultures are not sufficient to recapitulate the disease course. Even so, a very limited number of 3-D scaffold-based therapies have been developed, and future studies should focus on their development in order to gain more in-depth insight in disease pathogenesis [ 210 , 211 ].…”

Section: The Role Of Scaffolds In Developing Regenerative Therapiementioning

confidence: 99%

“…Even if performed in healthy cells, the model could prove to be relatable and applicable for the study of the pathogenesis of motor neuron diseases [ 212 ]. The possibility of combining healthy and diseased cells (such as the ones that can be obtained from ALS patients) allows the identification of the effects of neuronal or astrocyte toxicity in ALS and to create more representative disease models [ 211 ]. Another aspect of ALS pathology that needs to be investigated is the muscles’ role in the disease.…”

Section: The Role Of Scaffolds In Developing Regenerative Therapiementioning

confidence: 99%

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Advances in Tissue Engineering and Innovative Fabrication Techniques for 3-D-Structures: Translational Applications in Neurodegenerative Diseases

Rey

Barzaghini

Nardini

et al. 2020

Cells

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In the field of regenerative medicine applied to neurodegenerative diseases, one of the most important challenges is the obtainment of innovative scaffolds aimed at improving the development of new frontiers in stem-cell therapy. In recent years, additive manufacturing techniques have gained more and more relevance proving the great potential of the fabrication of precision 3-D scaffolds. In this review, recent advances in additive manufacturing techniques are presented and discussed, with an overview on stimulus-triggered approaches, such as 3-D Printing and laser-based techniques, and deposition-based approaches. Innovative 3-D bioprinting techniques, which allow the production of cell/molecule-laden scaffolds, are becoming a promising frontier in disease modelling and therapy. In this context, the specific biomaterial, stiffness, precise geometrical patterns, and structural properties are to be considered of great relevance for their subsequent translational applications. Moreover, this work reports numerous recent advances in neural diseases modelling and specifically focuses on pre-clinical and clinical translation for scaffolding technology in multiple neurodegenerative diseases.

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Section: The Role Of Scaffolds In Developing Regenerative Therapiementioning

confidence: 99%

Section: The Role Of Scaffolds In Developing Regenerative Therapiementioning

confidence: 99%

Advances in Tissue Engineering and Innovative Fabrication Techniques for 3-D-Structures: Translational Applications in Neurodegenerative Diseases

Rey

Barzaghini

Nardini

et al. 2020

Cells

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“…However, it remains elusive to discriminate the precise role of each cell type in the pathology. The use of in vitro models combining each cell type extracted from the SOD1 G93A and/or SOD1 WT mice, cultured individually or cocultured together, could be helpful to better understand the role of specific cellular type in the disease pathogenesis (Berthod & Gros‐Louis, 2012; Osaki et al, 2018). To be more informative, these cell combinations should be performed in a three‐dimensional (3D) environment, allowing cell‐to‐cell interaction, better mimicking the in vivo situation (Roy et al, 2020).…”

Section: Introductionmentioning

confidence: 99%

Tissue‐engineered in vitro modeling of the impact of Schwann cells in amyotrophic lateral sclerosis

Louit

Beaudet

Gros‐Louis

et al. 2022

Biotech & Bioengineering

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Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disease affecting upper and lower motor neurons (MNs). To investigate whether Schwann cells could be involved in the disease pathogenesis, we developed a tissueengineered three-dimensional (3D) in vitro model that combined MNs cocultured with astrocytes and microglia seeded on top of a collagen sponge populated with epineurium fibroblasts to enable 3D axonal migration. C2C12 myoblasts were seeded underneath the sponge in the presence or absence of Schwann cells. To reproduce an ALS cellular microenvironment, MNs, astrocytes, and microglia were extracted from SOD1 G93A mice recapitulating many aspects of the human disease.

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“…To assess the effects of the secretomes from the non-treated and anti-miR-124-treated mSOD1 MNs on microglia polarization, microglial cells were isolated from the SC of WT mice pups and maintained for 2 days in vitro (2DIV) after being 21 DIV in mixed glial culture [ 27 ]. To further evaluate the harmful (the first) and beneficial (the second) effects of such secretomes, we used SC organotypic cultures from mSOD1 mice at early symptomatic stage (10–12 weeks-old), which are considered the best way to preserve cell–cell interactions and the biochemical organization of cells in the SC [ 28 , 29 ].…”

Section: Introductionmentioning

confidence: 99%

Overexpression of miR-124 in Motor Neurons Plays a Key Role in ALS Pathological Processes

Vaz

Vizinha

Morais

et al. 2021

IJMS

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miRNA(miR)-124 is an important regulator of neurogenesis, but its upregulation in SOD1G93A motor neurons (mSOD1 MNs) was shown to associate with neurodegeneration and microglia activation. We used pre-miR-124 in wild-type (WT) MNs and anti-miR-124 in mSOD1 MNs to characterize the miR-124 pathological role. miR-124 overexpression in WT MNs produced a miRNA profile like that of mSOD1 MNs (high miR-125b; low miR-146a and miR-21), and similarly led to early apoptosis. Alterations in mSOD1 MNs were abrogated with anti-miR-124 and changes in their miRNAs mostly recapitulated by their secretome. Normalization of miR-124 levels in mSOD1 MNs prevented the dysregulation of neurite network, mitochondria dynamics, axonal transport, and synaptic signaling. Same alterations were observed in WT MNs after pre-miR-124 transfection. Secretome from mSOD1 MNs triggered spinal microglia activation, which was unno-ticed with that from anti-miR-124-modulated cells. Secretome from such modulated MNs, when added to SC organotypic cultures from mSOD1 mice in the early symptomatic stage, also coun-teracted the pathology associated to GFAP decrease, PSD-95 and CX3CL1-CX3CR1 signaling im-pairment, neuro-immune homeostatic imbalance, and enhanced miR-124 expression levels. Data suggest that miR-124 is implicated in MN degeneration and paracrine-mediated pathogenicity. We propose miR-124 as a new therapeutic target and a promising ALS biomarker in patient sub-populations.

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In Vivo and In Vitro Models to Study Amyotrophic Lateral Sclerosis

Cited by 8 publications

References 185 publications

Advances in Tissue Engineering and Innovative Fabrication Techniques for 3-D-Structures: Translational Applications in Neurodegenerative Diseases

Advances in Tissue Engineering and Innovative Fabrication Techniques for 3-D-Structures: Translational Applications in Neurodegenerative Diseases

Tissue‐engineered in vitro modeling of the impact of Schwann cells in amyotrophic lateral sclerosis

Overexpression of miR-124 in Motor Neurons Plays a Key Role in ALS Pathological Processes

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