Fibular muscular atrophy is a common genetic disease of the peripheral nervous system. With more than 2 million patients worldwide, it is considered a rare disease that is difficult to cure. Researchers from the Max Planck Institute for Experimental Medicine in Germany and the Medical Center of the University of G?ttingen have discovered that a harmless food supplement called lecithin can treat and improve peroneal muscle atrophy. The results of the research were published in Nature. Newsletter.
Due to a genetic defect, doubling of the gene pmp22 leads to progressive neurofibromuscular atrophy in patients, which is a slowly progressive nerve injury. Patients may have first symptoms such as difficulty walking or deformities of the feet as a child. Later, sensory disturbances such as limb numbness, tingling and pain may occur, and the strength of the legs and arms gradually diminishes. In severe cases, patients can only live in a wheelchair. So far, there is no cure for peroneal muscular atrophy, because its disease mechanism is unclear.
A membrane, called a myelin sheath, surrounds the axons of nerve cells and consists of Schwann cells and other types of neural support cells. Its role is to prevent electrical impulses from being transmitted from a neuron axon to another neuron axon. This time, the researchers used transgenic mouse experiments to find that the fat metabolism of the affected Schwann cells would be disturbed, and sufficient myelin sheaths could not be formed. In patients with peroneal muscular atrophy, it is because of the abnormal fat metabolism of Schwann cells that many nerve fibers have functional dysfunction without myelin.
Further research found that lecithin can promote fat production in damaged Schwann cells, thereby improving myelination. Lecithin is a so-called phospholipid mixture derived from soy or egg yolk and is a harmless food supplement. As one of the research projects funded by the German Federal Ministry of Education and Research, researchers for the first time through cell culture experiments in transgenic mice have shown that lecithin can be absorbed by Schwann cells and promote myelin sheath production. The director of the Max Planck Institute of Experimental Medicine, Dr. Stassart, said that through several studies of lecithin at different doses and treatment cycles in diseased rats, researchers found that phospholipid therapy can not only promote myelin formation, but also Regardless of how treatment is started, the disease process can be significantly reduced, and it has been proven that lecithin can be used as an adjuvant therapeutic agent for the treatment of peroneal muscle atrophy and other demyelinating diseases.