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Zebrafish Models of Autosomal Recessive Ataxias

Published version
Peer-reviewed

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Authors

Quelle-Regaldie, Ana  ORCID logo  https://orcid.org/0000-0003-0498-8741
Sobrido-Cameán, Daniel  ORCID logo  https://orcid.org/0000-0001-8239-2965
Barreiro-Iglesias, Antón  ORCID logo  https://orcid.org/0000-0002-7507-080X
Sobrido, María Jesús 

Abstract

Autosomal 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.

Description

Keywords

zebrafish, hereditary recessive ataxias, neurodegenerative disorders, genetic edition

Journal Title

Cells

Conference Name

Journal ISSN

2073-4409

Volume Title

10

Publisher

MDPI
Sponsorship
Instituto de Salud Carlos III (PI17/01582)