In vitro myogenesis activation of specific muscle-derived stem cells from patients with Duchenne muscular dystrophy

作者：Zhang, Hui-Li^*; Li, Ze; Cheng, Qiu-Sheng; Chen, Xi; Zhang, Cheng; Zeng, Tao^*

来源：Transplant Immunology, 2023, 77: 101796.

DOI：10.1016/j.trim.2023.101796

• Muscle derived stem cells
• Myogenicity
• Duchenne muscular dystrophy
• Myogenesis activation
• Pax7

Background: Muscle-derived stem cells (MDSCs) contribute to the repair of injured muscles. However, the myogenicity of MDSCs generated from patients with Duchenne muscular dystrophy (DMD) relative to healthy individuals remains unclear.Methods: A human DMD model was established using the stem cells prepared from muscle derived from patients with DMD (DMD-hMDSCs). The expression of myogenic lineage-specific markers in MDSCs was examined with immunofluorescence, real-time polymerase chain reaction, and western blotting.Results: It was demonstrated that, compared with cells from healthy subjects, DMD-hMDSCs are primed to self-differentiate in growth-inducing medium (GM) and robustly differentiate into myotubes in differentiation-inducing medium(DM). This feature was termed "myogenesis activation," and it was speculated that it con-tributes to the depletion of myogenic progenitors. Furthermore, MDSCs consistently express pax7, but the time-course of this expression does not correlate with the expression of the myogenic lineage-specific markers.Conclusions: The myogenesis activation in DMD-hMDSCs demonstrated in this study may provide novel mech-anistic insights into DMD pathogenesis and potential therapies.

• 单位
  1; 广州医学院; 中山大学