Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives

Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell autonomous neurodegenerative disease characterized by upper and lower motor neuron loss. Several genetic mutations lead to ALS development and many emerging gene mutations have been discovered in recent years. O...

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Autores principales:	Tiziana Bonifacino, Roberta Arianna Zerbo, Matilde Balbi, Carola Torazza, Giulia Frumento, Ernesto Fedele, Giambattista Bonanno, Marco Milanese
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	amyotrophic lateral sclerosis genetic animal models yeast worm fly zebrafish Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/b536ab023b774d06bda5488d372ea589
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spelling	oai:doaj.org-article:b536ab023b774d06bda5488d372ea5892021-11-25T17:54:34ZNearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives10.3390/ijms2222122361422-00671661-6596https://doaj.org/article/b536ab023b774d06bda5488d372ea5892021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/22/12236https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell autonomous neurodegenerative disease characterized by upper and lower motor neuron loss. Several genetic mutations lead to ALS development and many emerging gene mutations have been discovered in recent years. Over the decades since 1990, several animal models have been generated to study ALS pathology including both vertebrates and invertebrates such as yeast, worms, flies, zebrafish, mice, rats, guinea pigs, dogs, and non-human primates. Although these models show different peculiarities, they are all useful and complementary to dissect the pathological mechanisms at the basis of motor neuron degeneration and ALS progression, thus contributing to the development of new promising therapeutics. In this review, we describe the up to date and 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. Moreover, we highlight similarities, differences, advantages, and limitations, aimed at helping the researcher to select the most appropriate experimental animal model, when designing a preclinical ALS study.Tiziana BonifacinoRoberta Arianna ZerboMatilde BalbiCarola TorazzaGiulia FrumentoErnesto FedeleGiambattista BonannoMarco MilaneseMDPI AGarticleamyotrophic lateral sclerosisgenetic animal modelsyeastwormflyzebrafishBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 12236, p 12236 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	amyotrophic lateral sclerosis genetic animal models yeast worm fly zebrafish Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	amyotrophic lateral sclerosis genetic animal models yeast worm fly zebrafish Biology (General) QH301-705.5 Chemistry QD1-999 Tiziana Bonifacino Roberta Arianna Zerbo Matilde Balbi Carola Torazza Giulia Frumento Ernesto Fedele Giambattista Bonanno Marco Milanese Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives
description	Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell autonomous neurodegenerative disease characterized by upper and lower motor neuron loss. Several genetic mutations lead to ALS development and many emerging gene mutations have been discovered in recent years. Over the decades since 1990, several animal models have been generated to study ALS pathology including both vertebrates and invertebrates such as yeast, worms, flies, zebrafish, mice, rats, guinea pigs, dogs, and non-human primates. Although these models show different peculiarities, they are all useful and complementary to dissect the pathological mechanisms at the basis of motor neuron degeneration and ALS progression, thus contributing to the development of new promising therapeutics. In this review, we describe the up to date and 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. Moreover, we highlight similarities, differences, advantages, and limitations, aimed at helping the researcher to select the most appropriate experimental animal model, when designing a preclinical ALS study.
format	article
author	Tiziana Bonifacino Roberta Arianna Zerbo Matilde Balbi Carola Torazza Giulia Frumento Ernesto Fedele Giambattista Bonanno Marco Milanese
author_facet	Tiziana Bonifacino Roberta Arianna Zerbo Matilde Balbi Carola Torazza Giulia Frumento Ernesto Fedele Giambattista Bonanno Marco Milanese
author_sort	Tiziana Bonifacino
title	Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives
title_short	Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives
title_full	Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives
title_fullStr	Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives
title_full_unstemmed	Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives
title_sort	nearly 30 years of animal models to study amyotrophic lateral sclerosis: a historical overview and future perspectives
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/b536ab023b774d06bda5488d372ea589
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Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives

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