Zebrafish Models of Autosomal Recessive Ataxias

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 mult...

Full description

Saved in:
Bibliographic Details
Published in:Cells (Basel, Switzerland) Switzerland), 2021-04, Vol.10 (4), p.836
Main Authors: Quelle-Regaldie, Ana, Sobrido-Cameán, Daniel, Barreiro-Iglesias, Antón, Sobrido, María Jesús, Sánchez, Laura
Format: Article
Language:English
Subjects:
Animal models
Apoptosis
Ataxia
Cell cycle
Central nervous system
Danio rerio
Disease
DNA damage
Genes
genetic edition
Genetic engineering
Genetic screening
Genomes
Genotype & phenotype
hereditary recessive ataxias
Infertility
Kinases
Metabolism
Molecular modelling
Mutation
Neurodegeneration
Neurodegenerative diseases
neurodegenerative disorders
Neurons
Paralysis
Phenotypes
Radiation
Review
Zebrafish
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2073-4409
2073-4409
DOI:10.3390/cells10040836