Duchenne muscular dystrophy (DMD), the most common lethal genetic disorder in children, is an X-linked recessive muscle disease characterized by the absence of dystrophin at the sarcolemma of muscle fibers. The primary defect is the absence of dystrophin, a subsarcolemmal structural protein which plays an important role in maintaining the physical integrity of the muscle fibers membrane. Dystrophin, and utrophin, is part of a complex of proteins that links the internal cytoskeleton of muscle fibers to the extra cellular matrix. Up to now, no method can cure the disease.
Mdx mice were setup with C57/BL10 mice, whose exon 23 of dystrophin gene was, inserted a premature stop codon. By this, the dystrophin gene of mdx mice was knockout and dystrophin protein is absent. One of the other membrane proteins, utrophin exists. It is a wide used animal model for study DMD.
There have reported that bone marrow cells (BMCs) transplanted into dystrophic mice (mdx) express the dystrophic protein and regenerate skeletal muscle. However, the ability of BMCs expressed dytrophin protein is limited. If mice age is a variation that influences BMCs expressing dystrophic protein in mdx mice after transplanted. It was found that the expression ability of BMCs in younger mice are better and report as follow.
Chen S, Age may Influence Dystrophin Expression for Bone Marrow Cells Transplantation in mdx Mice
Last date updated on July, 2014