Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice

Martina Chrisam, Marinella Pirozzi, Silvia Castagnaro, Bert Blaauw, Roman Polishchuck, Francesco Cecconi, Paolo Grumati, Paolo Bonaldo

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)2142-2152
Number of pages11
JournalAutophagy
Volume11
Issue number12
DOIs
Publication statusPublished - Jan 1 2015

Keywords

  • Autophagy
  • Collagen VI
  • Muscular dystrophies
  • Skeletal muscle
  • Spermidine

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology

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