Limiting Protein Synthesis to Restore Myelination

This article describes a possible treatment for a hereditary disease that results from problems with myelinaiton. Charcot-Marie-Tooth (CMT) disease affects the nerves in the PNS and is a common hereditary neriological disorder. The disease results when the proteins that produce myelin misfold. When cells recognize that there has been a problem with protein folding, they drastically slow down protein production so the problem can be corrected. When protein synthesis needs to be turned back on, a protein named Gadd34 comes in. Gadd34, however, activates protein synthesis at a rate that is too high, resulting in a disruption of translational homeostasis. The results of the research show that if Gadd34 is reduced, myelination is actually improved. This means that the body actually benefits if protein synthesis is not turned on completely. In mouse models, salubrinal has been used to reduce Gadd34. Unfortunately, this research drug is not safe for human use, so the next step is to develop a derivative of salubrinal.


This article was interesting to me because it involved a lot of information that I learned in my neuroanatomy class this semester. The researchers believe that these findings can help the understanding of other diseases that are caused from misfolded proteins, like Alzheimer's diesease and Parkinson's disease. Although the treatment is not available for human use yet, any advancement in medicine is a step in the right direction.



http://www.sciencedaily.com/releases/2013/04/130426135037.htm