TY  - JOUR
A1  - Chrisam, Martina
A1  - Pirozzi, Marinella
A1  - Castagnaro, Silvia
A1  - Blaauw, Bert
A1  - Polishchuck, Roman
A1  - Cecconi, Francesco
A1  - Grumati, Paolo
A1  - Bonaldo, Paolo
T1  - Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice
T2  - Autophagy
N2  - 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.
KW  - autophagy
KW  - collagen VI
KW  - muscular dystrophies
KW  - skeletal muscle
KW  - spermidine
Y1  - 2015
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/41857
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-418577
SN  - 1554-8635
SN  - 1554-8627
N1  - This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0/), 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.
VL  - 11
IS  - 12
SP  - 2142
EP  - 2152
PB  - Taylor & Francis
CY  - Abingdon, Oxon
ER  -