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

Morphometric analysis confirmed a decreased percentage of aberrant mitochondria in muscles of spermidine-treated col6a1 −/− animals, which was significant after 30 mM per os treatment ( P = 0.039) whereas it almost reached significance after 50 mg/kg intraperitoneal treatment ( P = 0.077) ( Fig. 2D ).

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Of note, while the difference in the normalized force developed by wild-type and col6a1 −/− mice under standard conditions was highly significant, animals of the 2 genotypes displayed similar normalized force values after spermidine treatment ( Fig. 2F ; Fig.

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spermidine treatment led to a positive trend in normalized force in col6a1 −/− mice ( Fig.

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