Litcius/Paper detail

<i>Drosophila</i> carrying epilepsy-associated variants in the vitamin B6 metabolism gene <i>PNPO</i> display allele- and diet-dependent phenotypes

Wanhao Chi, Atulya Iyengar, Wenqin Fu, Wei Liu, Abigayle Berg, Chun-Fang Wu, Xiaoxi Zhuang

2022Proceedings of the National Academy of Sciences18 citationsDOIOpen Access PDF

Abstract

Significance Both genetic and environmental factors contribute to epilepsy. Understanding their contributions and interactions helps disease management. However, it is often challenging to study gene–environment interaction in humans due to their heterogeneous genetic background and less controllable environmental factors. The fruit fly, Drosophila melanogaster , has been proven to be a powerful model to study human diseases, including epilepsy. We generated knock-in flies carrying different epilepsy-associated pyridox(am)ine 5 ′ -phosphate oxidase ( PNPO ) alleles and studied the developmental, behavioral, electrophysiological, and fitness effects of each mutant allele under different dietary conditions. We showed that phenotypes in knock-in flies are allele and diet dependent, providing clues for timely and specific diet interventions. Our results offer biological insights into mechanisms underlying phenotypic variations and specific therapeutic strategies.

Topics & Concepts

AlleleEpilepsyPhenotypeBiologyGeneticsGeneNeuroscienceMetabolism and Genetic DisordersAmino Acid Enzymes and MetabolismRNA and protein synthesis mechanisms