Abstract

ABSTRACTFirst Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms, helping early-career researchers promote themselves alongside their papers. Hui-Ying Tsai and Shih-Cheng Wu are co-first authors on ‘Loss of the Drosophila branched-chain α-ketoacid dehydrogenase complex results in neuronal dysfunction’, published in DMM. Hui-Ying is a research assistant in the lab of Chun-Hong Chen at National Health Research Institutes, Zhunan, Taiwan. Her research interest is modeling the human neurological disease maple syrup urine disease in Drosophila, assessing behavior as well as brain damage. Shih-Cheng is a postdoc in the same lab, with interests in modeling human disease and immunometabolism.

Highlights

  • How would you explain the main findings of your paper to non-scientific family and friends? H-YT & S-CW: Amino acid metabolism is responsible for the body’s systemic homeostasis, which is dysregulated inherently in maple syrup urine disease (MSUD), which is attributed to abnormally excessive levels of branched-chain amino acids (BCAAs)

  • We developed a system using Drosophila with BCAA-catabolizing deficiency, potentially providing the ability to elucidate the pathogenesis of MSUD and aid the development of therapeutic methodology

  • In addition to recapitulating clinical manifestations, we showed that oxidative stress plays a potential role in modulating brain apoptosis in the dDBT mutant, and provide in vivo evidence that apoptosis and reactive oxygen species (ROS) are risk factors leading to neurological illness in our Drosophila model of MSUD

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Summary

Introduction

First person – Hui-Ying Tsai and Shih-Cheng Wu First Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms, helping early-career researchers promote themselves alongside their papers. Hui-Ying Tsai and Shih-Cheng Wu are co-first authors on ‘Loss of the Drosophila branched-chain α-ketoacid dehydrogenase complex results in neuronal dysfunction’, published in DMM.

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