Where and Why Modeling Amyotrophic Lateral Sclerosis
TLDR
In this paper, the authors present an updated and comprehensive review of how eukaryotic unicellular and multicellular organisms that reproduce a few of the main clinical features of ALS have helped in ALS research to dissect the pathological pathways of the disease insurgence and progression.Abstract:
Over the years, researchers have leveraged a host of different in vivo models in order to dissect amyotrophic lateral sclerosis (ALS), a neurodegenerative/neuroinflammatory disease that is heterogeneous in its clinical presentation and is multigenic, multifactorial and non-cell autonomous. These models include both vertebrates and invertebrates such as yeast, worms, flies, zebrafish, mice, rats, guinea pigs, dogs and, more recently, non-human primates. Despite their obvious differences and peculiarities, only the concurrent and comparative analysis of these various systems will allow the untangling of the causes and mechanisms of ALS for finally obtaining new efficacious therapeutics. However, harnessing these powerful organisms poses numerous challenges. In this context, we present here an updated and comprehensive review of how eukaryotic unicellular and multicellular organisms that reproduce a few of the main clinical features of the disease have helped in ALS research to dissect the pathological pathways of the disease insurgence and progression. We describe common features as well as discrepancies among these models, highlighting new insights and emerging roles for experimental organisms in ALS.read more
Citations
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Journal ArticleDOI
Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives.
Tiziana Bonifacino,Roberta Arianna Zerbo,Matilde Balbi,Carola Torazza,Giulia Frumento,Ernesto Fedele,Giambattista Bonanno,Marco Milanese +7 more
TL;DR: A review of the available ALS genetic animal models, classified by the different genetic mutations and divided per species, pointing out their features in modeling, the onset and progression of the pathology, as well as their specific pathological hallmarks as discussed by the authors.
Journal ArticleDOI
Fly for ALS: Drosophila modeling on the route to amyotrophic lateral sclerosis modifiers.
TL;DR: A review of ALS modifiers validated in C9ORF72, SOD1, FUS, TDP-43 and Ataxin-2 Drosophila models is presented in this article.
Journal ArticleDOI
Novel P2X7 Antagonist Ameliorates the Early Phase of ALS Disease and Decreases Inflammation and Autophagy in SOD1-G93A Mouse Model.
Savina Apolloni,Paola Fabbrizio,Susanna Amadio,Giulia Napoli,Mattia Freschi,Francesca Sironi,Paolo Pevarello,Paola Tarroni,Chiara Liberati,Caterina Bendotti,Cinzia Volonté +10 more
TL;DR: In this paper, the purinergic ionotropic P2X7 receptor plays a dual role in disease progression by acting at different cellular and molecular levels, and the Axxam proprietary P2x7 antagonist, AXX71, was tested in SOD1-G93A mice, by assessing some behavioral and molecular parameters, among which are disease progression, survival, gliosis and motor neuron wealth.
Journal ArticleDOI
Organ on a Chip: A Novel in vitro Biomimetic Strategy in Amyotrophic Lateral Sclerosis (ALS) Modeling
Babak Arjmand,Shayesteh Kokabi Hamidpour,Zahra Amini and Hossein Rabbani,Akram Tayanloo-Beik,Fakher Rahim,Hamid Reza Aghayan,Bagher Larijani +6 more
TL;DR: It can be concluded that lab on a chip can make a major contribution as a biomimetic micro-physiological system in the treatment of neurodegenerative disorders such as ALS.
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Boosting the peripheral immune response in the skeletal muscles improved motor function in ALS transgenic mice
Ronald J. Tinker,Ian Shrier +1 more
TL;DR: In this paper , a comparative study performed in fast and slow-progressing SOD1G93A mice spotlights the nature and temporal activation of the inflammatory response as limiting factors to preserve the periphery and interfere with the disease course.
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