The protein ZEB1 protects the skeletal muscle from acute and chronic injury and promotes its repair

What is it about?

Duchenne Muscular Dystrophy (DMD) is a genetic disease—caused by the modification of a gene—characterized by progressive loss of muscle, most commonly starting in childhood. The muscles of DMD patients suffer continuous damage but they are unable to repair themselves. Using muscle samples from DMD patients and an experimental model of DMD (the mdx mouse) we found that a protein named ZEB1 protect DMD muscles from damage and is required for their repair. Some cells of our immune system known as macrophages enter the dystrophic muscle and secrete substances that produce inflammation and damage the muscle. ZEB1 inhibits that macrophages produce this substance. In addition, in DMD muscles, the cells that are responsible to repair the muscle after damage, so-called muscle stem cells, are also defective. We found that ZEB1 is expressed in muscle stem cells and promotes muscle repair in DMD muscles.

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