| 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
|
| リソース情報 |
| 実験動物マウス |
RBRC00806 |
