DMD: altered iron metabolism in cardiomyocytes corrected by deferoxamine or pioglitazone

A Polish team has investigated the mechanism involved in Duchenne cardiomyopathy by studying the transcriptome and proteome of exonPartie codante de l’ADN au sein d’un gène. 50-deleted cardiomyocytesCellules musculaires cardiaques. derived from human iPS cells. She found :

• a decrease in mitoNEET protein levels ;
• an increase in labile iron in the cytoplasm and mitochondria;
• a decrease in ferroportin ;
• an increase in ferritin and transferrin receptor.

Correction of the mutationModification soudaine et transmissible du matériel génétique. Elle peut être spontanée ou induite par des agents dits » mutagènes » (radiations, produits toxiques,…). by CRISP/Cas9 restores normal iron levels in cardiomyocytes; treatment with deferoxamine or pioglitazone reduces free radicals in DMD cardiomyocytes.

Dysregulated iron homeostasis in dystrophin-deficient cardiomyocytes: correction by gene editing and pharmacological treatment. Andrysiak K, Machaj G, Priesmann D et al. Cardiovasc Res. 2023 Dec.