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Charcot Marie Tooth and Dejerine-Sottas syndrome are groups of diseases that involve the breakdown of the myelin sheath covering nerve axons.
As this myelin sheath breaks down, people who have these disorders suffer nerve damage in the arms and legs those with Dejerine-Sottas disease may never walk or may lose the ability to walk by the time they are teenagers.
Researchers have known that a protein called PMP22, which is important for nerve myelin, is likely involved in the disease. But because the protein is so small and part of the cell membrane, it s difficult to study. Now, an interdisciplinary team from the University of Michigan and Vanderbilt University have used a cutting-edge technique called ion mobility-mass spectrometry (IM-MS) to show that an unstable, mutant version of the PMP22 protein associates with another mutant PMP22, forming a stable complex called a dimer.