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

  • 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.

Download full text files

Export metadata

Additional Services

Share in Twitter Search Google Scholar
Author:Martina Chrisam, Marinella Pirozzi, Silvia Castagnaro, Bert Blaauw, Roman Polishchuck, Francesco CecconiORCiDGND, Paolo GrumatiORCiDGND, Paolo Bonaldo
Pubmed Id:
Parent Title (English):Autophagy
Publisher:Taylor & Francis
Place of publication:Abingdon, Oxon
Document Type:Article
Year of Completion:2015
Date of first Publication:2015/11/13
Publishing Institution:Universit├Ątsbibliothek Johann Christian Senckenberg
Release Date:2016/11/01
Tag:autophagy; collagen VI; muscular dystrophies; skeletal muscle; spermidine
Page Number:11
First Page:2142
Last Page:2152
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The moral rights of the named author(s) have been asserted.
Institutes:Biochemie, Chemie und Pharmazie / Biochemie und Chemie
Medizin / Medizin
Dewey Decimal Classification:5 Naturwissenschaften und Mathematik / 57 Biowissenschaften; Biologie / 570 Biowissenschaften; Biologie
6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Licence (German):License LogoCreative Commons - Namensnennung 3.0