Charcot-Marie-Tooth Disease: Theophylline Boosts Myelin Production in CMT1A Mice

According to a story from Charcot-Marie-Tooth News, a recent study using a mouse model of Charcot-Marie-Tooth disease type 1A produced some interesting results. The researchers found that administration of theophylline was able to increase myelination, the process by which myelin, a fatty layer which coats and protects nerve cells, is produced. In this form of Charcot-Marie-Tooth disease, along with many others, the layer of myelin is damaged.

About Charcot-Marie-Tooth Disease

Charcot-Marie-Tooth disease is a hereditary disorder of the peripheral nervous system. It is most characterized by a progressive loss of touch sensation and muscle tissue in several different parts of the body. The cause of this disease is usually linked to a genetic mutation, but the mutation involved varies depending on the variant of Charcot-Marie-Tooth disease. There are multiple types of Charcot-Marie-Tooth disease, with all types aside from type 2 having a demyelination effect. Symptoms include foot drop, muscle wasting (typically in the arms, legs, and hands), painful muscle spasms, loss of sensation in the limbs, scoliosis, trouble speaking, chewing, and swallowing, and tremors. Treatment typically includes therapy and surgery in order to maintain function. There is no cure. The disease can occur early in life or as late as the 30s and 40s. To learn more about Charcot-Marie-Tooth disease, click here.

Study Findings

Theophylline is typically used to treat respiratory illnesses such as asthma. The CMT1A mice were given either the medication or a saline solution at a low dose for four days. After 21 days, the coordination of the mice was tested. They found that the mice that had been treated with theophylline has better coordination than those that had not; however, there did not appear to be an improvement in muscle strength at the time that the mice were tested.

The scientists also noted that the large caliber nerve fibers of treated mice developed a thin myelin sheath, whereas in the untreated mice, the myelin was not present. Overall, the researchers concluded that theophylline should be subjected to further evaluation as a potential therapy in Charcot-Marie-Tooth disease type 1A and other peripheral neuropathies with a demyelinating effect.

You can find the full text of this study here in the scientific journal Biomedicines.

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