细胞疗法可改善mdx/SCID小鼠的骨骼肌组织病的病理机制

Cell Therapy Can Improve the Pathological Mechanism of Skeletal Myopathy in mdx/SCID Mice

杜氏肌营养不良症(duchenne muscular dystrophy,DMD)是一种X连锁隐性遗传病,能够导致严重的肌肉变性,和严重的肌营养不良症,表现出骨骼肌纤维的严重进行性退化,并导致患者死亡。本研究通过使用几种不同的方案研究了hDPSCs和hAPSCs向骨骼肌成肌谱系分化的潜力,以确定实现成肌承诺的最佳条件。同时为了验证h DPSCs和hAPSCs向骨骼肌成肌谱系的有效分化。我们研究了肌肉特异性因子和标记物的表达,包括肌生成素,肌球蛋白重链(MyHC)等。本研究通过使用几种不同的方案研究了hDPSCs和hAPSCs向骨骼肌成肌谱系分化的潜力,以确定实现成肌承诺的最佳条件。同时为了验证hDPSCs和hAPSCs向骨骼肌成肌谱系的有效分化,我们研究了肌肉特异性因子和标记物的表达,包括肌生成素,肌球蛋白重链(MyHC)等。本研究结果显示,植入mdx/SCID小鼠宿主肌肉中的两种细胞群都通过旁分泌作用促进了血管生成并减少了纤维化,最终导致营养不良性肌肉的组织病理学改善。因此本研究认为hAPSCs和hDPSCs代表干细胞的潜在来源的翻译策略,以提高组织病理学和潜在的缓解DMD患者的肌肉无力。

Duchenne muscular dystrophy(duchenne muscular dystrophy,DMD)is a kind of X-linked recessive genetic disease,which can lead to serious muscle degeneration,and serious muscular dystrophy,showing serious progressive degradation of skeletal muscle fibers,and leading to death of patients.The research used several different methods to study the differentiation potential of h DPSCs and h APSCs to myogenic lineage of skeletal muscle,so as to determine the best conditions for myogenic commitment.At the same time,in order to verify the effective differentiation of hDPSCs and hAPSCs to myogenic lineage of skeletal muscle.The research studied the expression of muscle specific factors and markers,including myogenin and myosin heavy chain(MyHC).The research used several different schemes to study the differentiation potential of hDPSCs and hAPSCs to myogenic lineage of skeletal muscle,so as to determine the best conditions to achieve myogenic commitment.At the same time,in order to verify the effective differentiation of hDPSCs and hAPSCs to myogenic lineage of skeletal muscle,we studied the expression of muscle specific factors and markers,including myogenin,myosin heavy chain(MyHC),etc.The results show that the two kinds of cell groups implanted into the host muscle of mdx/SCID mice promote angiogenesis and reduce fibrosis through paracrine effect,which eventually leads to the improvement of histopathology of malnourished muscles.Therefore,we believe that hAPSCs and hDPSCs represent the translation strategies of potential sources of stem cells to improve histopathology and potentially relieve muscle weakness in DMD patients.