A group of researchers in China have identified a protein that plays a role in the development of symptoms in an animal model of myasthenia gravis. By blocking the activity of the protein, the researchers prevented development of pathology in the animal model and believe this protein may represent a target for future therapeutics.
A group of researchers in China have identified that a protein called IL-9 plays a role in the development of symptoms in an animal model of myasthenia gravis. The researchers found that levels of the protein increased as signs of pathology (tissue damage) developed in rats, and when the actions of IL-9 were blocked development of the pathology slowed.
IL-9 is a cytokine - a type of protein used by the immune system to pass messages between cells - and it has a wide range of effects. It is able to activate many cells in the immune system including B-cells which can produce the antibodies that cause the pathology. The protein is produced by recently discovered TH9 cells, which researchers believe may also play a role in other conditions including asthma.
What does this mean for patients?
The work has identified a protein that plays a role in the development of the codnition in an animal model of myasthenia gravis. It is important to note that this work is at an early stage and the techniques that were used to prevent the IL-9 protein from working could not be used in the clinic. However, now that a potential target has been developed, researchers may be able to develop techniques that have the same effect in blocking IL-9 and that would be suitable for use in the clinic.
Myasthenia gravis is not an inherited disease and does not usually occur in families. It is caused by a malfunctioning of the immune system which can happen at any time in a person's life where the immune system instead of attacking infections such as viruses, mistakenly attacks proteins in the body. In myasthenia gravis the immune system specifically attacks proteins on the muscle called 'acetylcholine receptors' (AChRs) which normally receive the signals from nerves which tell the muscles to contract. This causes weakness and excessive muscle fatigue.Symptoms include weakness in the arms and legs, chronic muscle fatigue, difficulty breathing, chewing and swallowing, slurred speech, droopy eyelids and blurred or double vision.
Current treatments for the condition rely on drugs that stop the immune system working properly. These are sometimes associated with side effects and researchers are now trying to develop new potential therapies that can target individual components of the immune system to potentially make effective drugs with reduced side effects.
The congenital myasthenic syndromes by comparison are a set of inherited disorders with a common symptom of muscle weakness and fatigue. Symptoms usually become apparent in the first two years of life. Individuals with congenital myasthenic syndromes carry mutations in genes that affect AChRs in different ways.
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