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dc.contributor.authorQuelle Regaldie, Ana
dc.contributor.authorSobrido Cameán, Daniel
dc.contributor.authorBarreiro Iglesias, Antón
dc.contributor.authorSobrido Gómez, María Jesús
dc.contributor.authorSánchez Piñón, Laura Elena
dc.date.accessioned2021-05-04T12:45:20Z
dc.date.available2021-05-04T12:45:20Z
dc.date.issued2021
dc.identifier.citationCells 2021, 10(4), 836; https://doi.org/10.3390/cells10040836
dc.identifier.urihttp://hdl.handle.net/10347/26109
dc.description.abstractAutosomal recessive ataxias are much less well studied than autosomal dominant ataxias and there are no clearly defined systems to classify them. Autosomal recessive ataxias, which are characterized by neuronal and multisystemic features, have significant overlapping symptoms with other complex multisystemic recessive disorders. The generation of animal models of neurodegenerative disorders increases our knowledge of their cellular and molecular mechanisms and helps in the search for new therapies. Among animal models, the zebrafish, which shares 70% of its genome with humans, offer the advantages of being small in size and demonstrating rapid development, making them optimal for high throughput drug and genetic screening. Furthermore, embryo and larval transparency allows to visualize cellular processes and central nervous system development in vivo. In this review, we discuss the contributions of zebrafish models to the study of autosomal recessive ataxias characteristic phenotypes, behavior, and gene function, in addition to commenting on possible treatments found in these models. Most of the zebrafish models generated to date recapitulate the main features of recessive ataxias
dc.description.sponsorshipThis research was funded by Fondo de Investigaciones Sanitarias-Instituto de Salud Carlos III (Spain), grant number: PI17/01582 and by the Asociación Galega de Ataxia (AGA)
dc.language.isoeng
dc.publisherMDPI
dc.rights© 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/)
dc.rightsAtribución 4.0 Internacional
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.subjectZebrafish
dc.subjectHereditary recessive ataxias
dc.subjectNeurodegenerative disorders
dc.subjectGenetic edition
dc.titleZebrafish Models of Autosomal Recessive Ataxias
dc.typeinfo:eu-repo/semantics/article
dc.identifier.DOI10.3390/cells10040836
dc.relation.publisherversionhttps://doi.org/10.3390/cells10040836
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.identifier.e-issn2073-4409
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess
dc.contributor.affiliationUniversidade de Santiago de Compostela. Departamento de Bioloxía Funcional
dc.contributor.affiliationUniversidade de Santiago de Compostela. Departamento de Zooloxía, Xenética e Antropoloxía Física
dc.description.peerreviewedSI


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© 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/)
Except where otherwise noted, this item's license is described as  © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/)





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