RT Journal Article
T1 PITX2 Enhances the Regenerative Potential of Dystrophic Skeletal Muscle Stem Cells.
A1 Vallejo, Daniel
A1 Hernández-Torres, Francisco
A1 Lozano-Velasco, Estefanía
A1 Rodriguez-Outeiriño, Lara
A1 Carvajal, Alejandra
A1 Creus, Carlota
A1 Franco, Diego
A1 Aránega, Amelia Eva
K1 PITX2
K1 miR-31
K1 muscle stem cells
K1 muscular dystrophy
AB Duchenne muscular dystrophy (DMD), one of the most lethal genetic disorders, involves progressive muscle degeneration resulting from the absence of DYSTROPHIN. Lack of DYSTROPHIN expression in DMD has critical consequences in muscle satellite stem cells including a reduced capacity to generate myogenic precursors. Here, we demonstrate that the c-isoform of PITX2 transcription factor modifies the myogenic potential of dystrophic-deficient satellite cells. We further show that PITX2c enhances the regenerative capability of mouse DYSTROPHIN-deficient satellite cells by increasing cell proliferation and the number of myogenic committed cells, but importantly also increasing dystrophin-positive (revertant) myofibers by regulating miR-31. These PITX2-mediated effects finally lead to improved muscle function in dystrophic (DMD/mdx) mice. Our studies reveal a critical role for PITX2 in skeletal muscle repair and may help to develop therapeutic strategies for muscular disorders.
YR 2018
FD 2018
LK http://hdl.handle.net/10668/12333
UL http://hdl.handle.net/10668/12333
LA en
DS RISalud
RD Apr 11, 2025