In Vitro and in Vivo Models of Amyotrophic Lateral Sclerosis: An Updated Overview

Gois, Auderlan M.; Mendonça, Deise M. F.; Freire, Marco Aurélio M.; Santos, José R.

doi:10.1016/j.brainresbull.2020.03.012

Cited by 42 publications

(44 citation statements)

References 177 publications

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“…The zebrafish presents many features of Amyotrophic lateral sclerosis (ALS), including decreased adult swimming endurance, loss of motor neurons, paralysis and larvael and adult neuromuscular junction defects (36)(37)(38)(39). Currently, several studies have been developed in zebrafish to understand the aetiology of ALS, through microinjection of cDNA or mRNA (18,37,39,40) and techniques such as zinc-finger nucleases (ZFNs), transcription activator-like effector nucleases (TALENs) and CRISPR/Cas9 that allow easy testing in Zebrafish the loss or overexpression of genetic function associated with ALS (18,38). Most of these studies focused on genes such as TAR DNA Binding Protein (TARDBP), FUS RNA binding protein (FUS) and chromosome 9 open reading frame 72 (C9orf72) (36)(37)(38)40).…”

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

“…With the development of transgenic zebrafish for ALS, it was possible to study neuronal toxicity associated with overexpression of Cu/Zn superoxide dismutase (SOD1). In zebrafish model, the SOD1 mutation demonstrated locomotor deficiency, paralysis, changes in nerve and muscle terminals, muscle atrophy, oxidative stress sensitivity and loss of motor neurons (37)(38)(39). Studies in Zebrafish using the TAR 43 protein (TDP-43) (34,36,37,39) suggested paralysis, neurodegeneration, oxidative stress in nerves and consequent deficiency of locomotion (18,23,37,38).…”

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

“…In zebrafish model, the SOD1 mutation demonstrated locomotor deficiency, paralysis, changes in nerve and muscle terminals, muscle atrophy, oxidative stress sensitivity and loss of motor neurons (37)(38)(39). Studies in Zebrafish using the TAR 43 protein (TDP-43) (34,36,37,39) suggested paralysis, neurodegeneration, oxidative stress in nerves and consequent deficiency of locomotion (18,23,37,38). The injection of human mutant FUS mRNA into Zebrafish resulted in the accumulation of cytosolic tension granules, aggregation of proteins in the cytoplasm of motor neurons, damage in the synaptic transmission of the neuromuscular junction, locomotor deficits and ventral root projection anomalies (18,36,37).…”

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

“…A repeated expression of the hexanucleotide "GGGCC" in the non-coding region of the C9ORF72 gene was also studied in zebrafish associated with different forms of ALS such as behavioural and cellular deficits related to locomotion and motor axonopathy (25,(37)(38)(39).…”

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

“…Kidney Disease 5,114,125,132 L Laboratórios 5,7,37,38,39,40,41,42 M Manual de Laboratório 37 Maracujazeiro 8,82,83,84 Medicamentos 14,33,34,35,72 Método de extração 5,7,60,61 Micropropagação 5,82 Mitose 5,1,2,3,4,5,7,8,9,11,136 Morfologia dos frutos 23…”

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Alterações Genotóxicas, Citotóxicas E Mutagênicas: Um Conteúdo a Ser Ilustrado E Trabalhado No Ensino Médio

Brito¹,

Chagas²,

Messias³

et al. 2021

A Genética E a Construção De Novos Paradigmas Nas Ciências Da Vida

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Direitos para esta edição cedidos à Atena Editora pelos autores. Todo o conteúdo deste livro está licenciado sob uma Licença de Atribuição Creative Commons. Atribuição-Não-Comercial-NãoDerivativos 4.0 Internacional (CC BY-NC-ND 4.0). O conteúdo dos artigos e seus dados em sua forma, correção e confiabilidade são de responsabilidade exclusiva dos autores, inclusive não representam necessariamente a posição oficial da Atena Editora. Permitido o download da obra e o compartilhamento desde que sejam atribuídos créditos aos autores, mas sem a possibilidade de alterá-la de nenhuma forma ou utilizá-la para fins comerciais. Todos os manuscritos foram previamente submetidos à avaliação cega pelos pares, membros do Conselho Editorial desta Editora, tendo sido aprovados para a publicação com base em critérios de neutralidade e imparcialidade acadêmica. A Atena Editora é comprometida em garantir a integridade editorial em todas as etapas do processo de publicação, evitando plágio, dados ou resultados fraudulentos e impedindo que interesses financeiros comprometam os padrões éticos da publicação. Situações suspeitas de má conduta científica serão investigadas sob o mais alto padrão de rigor acadêmico e ético.

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Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

Section: Amyotrophic Lateral Sclerosis In the Zebrafish Modelmentioning

confidence: 99%

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Alterações Genotóxicas, Citotóxicas E Mutagênicas: Um Conteúdo a Ser Ilustrado E Trabalhado No Ensino Médio

Brito¹,

Chagas²,

Messias³

et al. 2021

A Genética E a Construção De Novos Paradigmas Nas Ciências Da Vida

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A NSC‐34 cell line‐derived spheroid model: Potential and challenges for in vitro evaluation of neurodegeneration

Arnaldi,

Casarotto,

Relucenti

et al. 2024

Microscopy Res & Technique

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Three‐dimensional (3D) spheroid models aim to bridge the gap between traditional two‐dimensional (2D) cultures and the complex in vivo tissue environment. These models, created by self‐clustering cells to mimic a 3D environment with surrounding extracellular framework, provide a valuable research tool. The NSC‐34 cell line, generated by fusing mouse spinal cord motor neurons and neuroblastoma cells, is essential for studying neurodegenerative diseases like amyotrophic lateral sclerosis (ALS), where abnormal protein accumulation, such as TAR‐DNA‐binding protein 43 (TDP‐43), occurs in affected nerve cells. However, NSC‐34 behavior in a 3D context remains underexplored, and this study represents the first attempt to create a 3D model to determine its suitability for studying pathology. We generated NSC‐34 spheroids using a nonadhesive hydrogel‐based template and characterized them for 6 days. Light microscopy revealed that NSC‐34 cells in 3D maintained high viability, a distinct round shape, and forming stable membrane connections. Scanning electron microscopy identified multiple tunnel‐like structures, while ultrastructural analysis highlighted nuclear bending and mitochondria alterations. Using inducible GFP‐TDP‐43‐expressing NSC‐34 spheroids, we explored whether 3D structure affected TDP‐43 expression, localization, and aggregation. Spheroids displayed nuclear GFP‐TDP‐43 expression, albeit at a reduced level compared with 2D cultures and generated both TDP‐35 fragments and TDP‐43 aggregates. This study sheds light on the distinctive behavior of NSC‐34 in 3D culture, suggesting caution in the use of the 3D model for ALS or TDP‐43 pathologies. Yet, it underscores the spheroids' potential for investigating fundamental cellular mechanisms, cell adaptation in a 3D context, future bioreactor applications, and drug penetration studies.Research Highlights 3D spheroid generation: NSC‐34 spheroids, developed using a hydrogel‐based template, showed high viability and distinct shapes for 6 days. Structural features: advanced microscopy identified tunnel‐like structures and nuclear and mitochondrial changes in the spheroids. Protein dynamics: the study observed how 3D structures impact TDP‐43 behavior, with altered expression but similar aggregation patterns to 2D cultures. Research implications: this study reveals the unique behavior of NSC‐34 in 3D culture, suggests a careful approach to use this model for ALS or TDP‐43 pathologies, and highlights its potential in cellular mechanism research and drug testing applications.

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Neuromuscular Junction-on-a-Chip for Amyotrophic Lateral Sclerosis Modeling

Alavi-Moghadam

Kokabi-Hamidpour

Rezaei‐Tavirani

et al. 2023

Methods in Molecular Biology

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In Vitro and in Vivo Models of Amyotrophic Lateral Sclerosis: An Updated Overview

Cited by 42 publications

References 177 publications

Alterações Genotóxicas, Citotóxicas E Mutagênicas: Um Conteúdo a Ser Ilustrado E Trabalhado No Ensino Médio

Alterações Genotóxicas, Citotóxicas E Mutagênicas: Um Conteúdo a Ser Ilustrado E Trabalhado No Ensino Médio

A NSC‐34 cell line‐derived spheroid model: Potential and challenges for in vitro evaluation of neurodegeneration

Neuromuscular Junction-on-a-Chip for Amyotrophic Lateral Sclerosis Modeling

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