Drosophila as a diet discovery tool for treating amino acid disorders

A defined diet for pre-adultDrosophila melanogaster

“…For example, human disorders such as Phenylketonuria manifest with rapid neurological decline prior to adulthood mitigated by a custom diet with single aa restriction that drastically improves health outcomes 9 . However, the majority of metabolic disorders lack therapeutic diets 8 . To demonstrate the potential of our diet as a tool to find new dietary treatments, we applied it in a model of human Branched Chain Amino Acid (BCAA) Transaminase ( BCAT2 ) deficiency, a rare disorder associated with developmental delay, intellectual disability, seizures, and movement defects 10 .…”

“…1j), restored fat levels, and enhanced body mass, without compromising adult lifespan. A synthetic diet on which Drosophila can be raised is particularly valuable in the modelling of human metabolic disorders, where phenotypic abnormalities often manifest prior to adulthood 8 . For example, human disorders such as Phenylketonuria manifest with rapid neurological decline prior to adulthood mitigated by a custom diet with single aa restriction that drastically improves health outcomes 9 .…”