Drosophila melanogaster as a Model to Study Fragile X-Associated Disorders.
Genes (Basel)
; 14(1)2022 12 28.
Article
in En
| MEDLINE
| ID: mdl-36672829
ABSTRACT
Fragile X syndrome (FXS) is a global neurodevelopmental disorder caused by the expansion of CGG trinucleotide repeats (≥200) in the Fragile X Messenger Ribonucleoprotein 1 (FMR1) gene. FXS is the hallmark of Fragile X-associated disorders (FXD) and the most common monogenic cause of inherited intellectual disability and autism spectrum disorder. There are several animal models used to study FXS. In the FXS model of Drosophila, the only ortholog of FMR1, dfmr1, is mutated so that its protein is missing. This model has several relevant phenotypes, including defects in the circadian output pathway, sleep problems, memory deficits in the conditioned courtship and olfactory conditioning paradigms, deficits in social interaction, and deficits in neuronal development. In addition to FXS, a model of another FXD, Fragile X-associated tremor/ataxia syndrome (FXTAS), has also been established in Drosophila. This review summarizes many years of research on FXD in Drosophila models.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Drosophila Proteins
/
Autism Spectrum Disorder
/
Fragile X Syndrome
Type of study:
Risk_factors_studies
Limits:
Animals
Language:
En
Journal:
Genes (Basel)
Year:
2022
Document type:
Article