| RRC ID |
79053
|
| 著者 |
Martelli F, Lin J, Mele S, Imlach W, Kanca O, Barlow CK, Paril J, Schittenhelm RB, Christodoulou J, Bellen HJ, Piper MDW, Johnson TK.
|
| タイトル |
Identifying potential dietary treatments for inherited metabolic disorders using Drosophila nutrigenomics.
|
| ジャーナル |
Cell Rep
|
| Abstract |
Inherited metabolic disorders are a group of genetic conditions that can cause severe neurological impairment and child mortality. Uniquely, these disorders respond to dietary treatment; however, this option remains largely unexplored because of low disorder prevalence and the lack of a suitable paradigm for testing diets. Here, we screened 35 Drosophila amino acid disorder models for disease-diet interactions and found 26 with diet-altered development and/or survival. Using a targeted multi-nutrient array, we examine the interaction in a model of isolated sulfite oxidase deficiency, an infant-lethal disorder. We show that dietary cysteine depletion normalizes their metabolic profile and rescues development, neurophysiology, behavior, and lifelong fly survival, thus providing a basis for further study into the pathogenic mechanisms involved in this disorder. Our work highlights the diet-sensitive nature of metabolic disorders and establishes Drosophila as a valuable tool for nutrigenomic studies for informing potential dietary therapies.
|
| 巻・号 |
43(3)
|
| ページ |
113861
|
| 公開日 |
2024-3-26
|
| DOI |
10.1016/j.celrep.2024.113861
|
| PII |
S2211-1247(24)00189-X
|
| PMID |
38416643
|
| PMC |
PMC11037929
|
| MeSH |
Amino Acid Metabolism, Inborn Errors*
Animals
Child
Diet
Drosophila
Humans
Infant
Metabolic Diseases* / genetics
Nutrigenomics
|
| IF |
8.109
|
| リソース情報 |
| ショウジョウバエ |
CAS-0001 |
