Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 8, Pages -Publisher
MDPI
DOI: 10.3390/ijms22083977
Keywords
amyotrophic lateral sclerosis; animal modeling; Saccharomyces cerevisiae; Caenorhabditis elegans; Drosophila melanogaster; Danio rerio; rodents; canines; non-human primates
Funding
- Italian Ministry of Health through Fondazione Santa Lucia IRCCS Ricerca Corrente
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Researchers have utilized a variety of in vivo models to dissect ALS, including both vertebrates and invertebrates. Concurrent and comparative analysis of these systems is essential to unravel the causes and mechanisms of ALS. These studies have helped to dissect the pathological pathways of ALS.
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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