Ontology highlight
ABSTRACT:
SUBMITTER: Reggio A
PROVIDER: S-EPMC7003708 | biostudies-literature | 2020 Mar
REPOSITORIES: biostudies-literature
Reggio Alessio A Rosina Marco M Krahmer Natalie N Palma Alessandro A Petrilli Lucia Lisa LL Maiolatesi Giuliano G Massacci Giorgia G Salvatori Illari I Salvatori Illari I Valle Cristiana C Testa Stefano S Gargioli Cesare C Fuoco Claudia C Castagnoli Luisa L Cesareni Gianni G Sacco Francesca F
Life science alliance 20200204 3
In Duchenne muscular dystrophy (DMD), the absence of the dystrophin protein causes a variety of poorly understood secondary effects. Notably, muscle fibers of dystrophic individuals are characterized by mitochondrial dysfunctions, as revealed by a reduced ATP production rate and by defective oxidative phosphorylation. Here, we show that in a mouse model of DMD (<i>mdx</i>), fibro/adipogenic progenitors (FAPs) are characterized by a dysfunctional mitochondrial metabolism which correlates with inc ...[more]