Where and Why Modeling Amyotrophic Lateral Sclerosis |
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Authors: | Francesco Liguori,Susanna Amadio,Cinzia Volont |
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Affiliation: | 1.Preclinical Neuroscience, IRCCS Santa Lucia Foundation, 00143 Rome, Italy; (F.L.); (S.A.);2.Institute for Systems Analysis and Computer Science “A. Ruberti”, National Research Council (IASI—CNR), 00185 Rome, Italy |
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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. |
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Keywords: | amyotrophic lateral sclerosis animal modeling Saccharomyces cerevisiae Caenorhabditis elegans Drosophila melanogaster Danio rerio rodents canines non-human primates |
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