論文 - 詳細
| RRC ID | 35985 |
|---|---|
| 著者 | Chrisam M, Pirozzi M, Castagnaro S, Blaauw B, Polishchuck R, Cecconi F, Grumati P, Bonaldo P. |
| タイトル | Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice. |
| ジャーナル | Autophagy |
| Abstract |
Autophagy is a self-degradative process responsible for the clearance of damaged or unnecessary cellular components. We have previously found that persistence of dysfunctional organelles due to autophagy failure is a key event in the pathogenesis of COL6/collagen VI-related myopathies, and have demonstrated that reactivation of a proper autophagic flux rescues the muscle defects of Col6a1-null (col6a1(-/-)) mice. Here we show that treatment with spermidine, a naturally occurring nontoxic autophagy inducer, is beneficial for col6a1(-/-) mice. Systemic administration of spermidine in col6a1(-/-) mice reactivated autophagy in a dose-dependent manner, leading to a concurrent amelioration of the histological and ultrastructural muscle defects. The beneficial effects of spermidine, together with its being easy to administer and the lack of overt side effects, open the field for the design of novel nutraceutical strategies for the treatment of muscle diseases characterized by autophagy impairment. |
| 巻・号 | 11(12) |
| ページ | 2142-52 |
| 公開日 | 2015-1-1 |
| DOI | 10.1080/15548627.2015.1108508 |
| PMID | 26565691 |
| PMC | PMC4835186 |
| MeSH | Animals Apoptosis / genetics Apoptosis / physiology Autophagy / genetics* Autophagy / physiology Collagen Type VI / deficiency* Disease Models, Animal Mice, Inbred C57BL Mice, Knockout Mitochondria / pathology Muscles / metabolism* Muscles / pathology Muscular Diseases / pathology* Spermidine / metabolism* |
| IF | 9.77 |
| 引用数 | 30 |
| WOS 分野 | CELL BIOLOGY |
| オルトメトリクス指標 |
オルトメトリクス指標項目
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| 最多言及媒体 | X(Twitter) |
| 各媒体での言及数の合計 | 4 |
| 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 | |
| 実験動物マウス | RBRC00806 |