Nedachi Takaki, Kawasaki Haruhisa, Inoue Eiji, Suzuki Takahiro, Nakagawa-Yagi Yuzo, Ishida Norio
Hakuju Institute for Health Science Co., Ltd., 1-37-5 Tomigaya, Shibuya-ku, Tokyo, 151-0063, Japan.
Institute for Chronobiology, Foundation for Advancement of International Science (FAIS), 3-24-16 Kasuga, Tsukuba, Ibaraki, 305-0812, Japan.
Biochem Biophys Rep. 2025 Jan 10;41:101915. doi: 10.1016/j.bbrep.2025.101915. eCollection 2025 Mar.
Gaucher's disease (GD) is a genetic disease characterized by a mutation in the metabolic enzyme glucocerebrosidase (GBA1), leading to the accumulation of glucosylceramide in tissues. We previously discovered that a -inserted mutation in the gene of fruit flies, , mimics human neuronopathic GD (nGD) characteristics, providing a promising model for studying the molecular mechanisms of the disease. We also reported that extremely low-frequency electric fields (ELF-EFs) promote sleep and extend the lifespan of wild-type flies. In this study, we show that ELF-EFs have health-promoting effects on nGD model flies. Firstly, the total sleep time and sleep episode duration of EF-exposed nGD model flies increased. EFs also extended the lifespans of nGD model flies. Additionally, the expression of the endoplasmic reticulum stress-related gene and autophagy-related gene were elevated after EF exposure. The effects of EF exposure on nGD flies are associated with the change of these genes expression. Our findings suggest that EF exposure may be effective as an additional therapy for nGD.
戈谢病(GD)是一种遗传性疾病,其特征是代谢酶葡萄糖脑苷脂酶(GBA1)发生突变,导致葡萄糖神经酰胺在组织中蓄积。我们之前发现,果蝇基因中的一个插入突变模仿了人类神经元型戈谢病(nGD)的特征,为研究该疾病的分子机制提供了一个有前景的模型。我们还报道过,极低频电场(ELF-EFs)可促进野生型果蝇的睡眠并延长其寿命。在本研究中,我们表明ELF-EFs对nGD模型果蝇具有促进健康的作用。首先,暴露于电场的nGD模型果蝇的总睡眠时间和睡眠片段时长增加。电场也延长了nGD模型果蝇的寿命。此外,内质网应激相关基因和自噬相关基因的表达在电场暴露后升高。电场暴露对nGD果蝇的影响与这些基因表达的变化有关。我们的研究结果表明,电场暴露可能作为nGD的一种辅助治疗有效。