Biomedical Research

Duchenne muscular dystrophy (DMD) is a severe recessive X-linked form of muscular dystrophy caused by mutations in the DMD gene located on the X chromosome,(Xp21). It is characterized by rapid progression of muscle degeneration, eventually leading to loss of ambulation and death. Dystrophin is the predominant DMD transcript expressed in striated muscle. DMD gene mutations, deletions or duplications most frequently result in loss of dystrophin expression in the muscle of these patients. Deletions are most commonly located in the 5' end and in the central region of the exons 44 to 52, of the gene. Application of Bone marrow derived mesenchymal stem cells represent a promising approach for supporting new clinical concepts of cell based therapy in regenerative medicine. The non hematopoietic component of bone marrow includes multipotent mesenchymal stem cells capable of differentiating into fat, bone, muscle, cartilage and endothelium. The present study was undertaken to assess the expression of Myogenin and CD105 in autologous bone marrow derived mesenchymal stromal cells of Duchenne Muscular Dystrophy patients. Ten DMD patients between 12 yrs to 24 yrs and 10 healthy controls were enrolled. With informed consent the bone marrow mononuclear cells were harvested, cultured and assessed for the expression of Myogenin and CD105 using Flourescence Activated Cell Sorter (FACS analysis). A significant increase in expression of Myogenin and CD105 was observed in the cultured autologous bone marrow derived Mesenchymal stromal cells of Duchenne Muscular Dystrophy patients. The expression of the above mesenchymal stem cell markers in patients with Duchenne Muscular Dystrophy indicate that autologous transplantation of bone marrow derived mesenchymal stromal cells could be of therapeutic potential.

Author(s): Sumit Jhajharia, Tapaswini Pradhan Ashok Kumar Das, Prakash C.Mohapatra
Abstract | Full-Text | PDF

Share this