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Zebrafish Models of Autosomal Dominant Ataxias.


ABSTRACT: Hereditary dominant ataxias are a heterogeneous group of neurodegenerative conditions causing cerebellar dysfunction and characterized by progressive motor incoordination. Despite many efforts put into the study of these diseases, there are no effective treatments yet. Zebrafish models are widely used to characterize neuronal disorders due to its conserved vertebrate genetics that easily support genetic edition and their optic transparency that allows observing the intact CNS and its connections. In addition, its small size and external fertilization help to develop high throughput assays of candidate drugs. Here, we discuss the contributions of zebrafish models to the study of dominant ataxias defining phenotypes, genetic function, behavior and possible treatments. In addition, we review

SUBMITTER: Quelle-Regaldie A 

PROVIDER: S-EPMC7922657 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature