RT Journal Article
T1 Zebrafish Models of Autosomal Recessive Ataxias
A1 Quelle Regaldie, Ana
A1 Sobrido Cameán, Daniel
A1 Barreiro Iglesias, Antón
A1 Sobrido Gómez, María Jesús
A1 Sánchez Piñón, Laura
K1 Zebrafish
K1 Hereditary recessive ataxias
K1 Neurodegenerative disorders
K1 Genetic edition
AB 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
PB MDPI
YR 2021
FD 2021
LK http://hdl.handle.net/10347/26109
UL http://hdl.handle.net/10347/26109
LA eng
NO Cells 2021, 10(4), 836; https://doi.org/10.3390/cells10040836
NO This 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)
DS Minerva
RD 24 abr 2026