論文 - 詳細
| RRC ID | 76330 |
|---|---|
| 著者 | Wu X, Nagasawa S, Muto K, Ueda M, Suzuki C, Abe T, Higashitani A. |
| タイトル | Mitochonic Acid 5 Improves Duchenne Muscular Dystrophy and Parkinson's Disease Model of Caenorhabditis elegans. |
| ジャーナル | Int J Mol Sci |
| Abstract |
Mitochonic Acid 5 (MA-5) enhances mitochondrial ATP production, restores fibroblasts from mitochondrial disease patients and extends the lifespan of the disease model "Mitomouse". Additionally, MA-5 interacts with mitofilin and modulates the mitochondrial inner membrane organizing system (MINOS) in mammalian cultured cells. Here, we used the nematode Caenorhabditis elegans to investigate whether MA-5 improves the Duchenne muscular dystrophy (DMD) model. Firstly, we confirmed the efficient penetration of MA-5 in the mitochondria of C. elegans. MA-5 also alleviated symptoms such as movement decline, muscular tone, mitochondrial fragmentation and Ca2+ accumulation of the DMD model. To assess the effect of MA-5 on mitochondria perturbation, we employed a low concentration of rotenone with or without MA-5. MA-5 significantly suppressed rotenone-induced mitochondria reactive oxygen species (ROS) increase, mitochondrial network fragmentation and nuclear destruction in body wall muscles as well as endogenous ATP levels decline. In addition, MA-5 suppressed rotenone-induced degeneration of dopaminergic cephalic (CEP) neurons seen in the Parkinson's disease (PD) model. Furthermore, the application of MA-5 reduced mitochondrial swelling due to the immt-1 null mutation. These results indicate that MA-5 has broad mitochondrial homing and MINOS stabilizing activity in metazoans and may be a therapeutic agent for these by ameliorating mitochondrial dysfunction in DMD and PD. |
| 巻・号 | 23(17) |
| 公開日 | 2022-8-24 |
| DOI | 10.3390/ijms23179572 |
| PII | ijms23179572 |
| PMID | 36076995 |
| PMC | PMC9455831 |
| MeSH | Adenosine Triphosphate Animals Caenorhabditis elegans / genetics Humans Indoleacetic Acids Mammals Muscular Dystrophy, Duchenne* / drug therapy Muscular Dystrophy, Duchenne* / genetics Parkinson Disease* / drug therapy Phenylbutyrates Rotenone / pharmacology |
| オルトメトリクス指標 |
オルトメトリクス指標項目
|
| 最多言及媒体 | X(Twitter) |
| 各媒体での言及数の合計 | 13 |
| 過去6か月間でのオルトメトリクス指標の変動値 | 0.0 |
| リソース情報 | |
| 線虫 | tm1730 |