Studying fruit flies, Botas and his colleagues found two RNA-binding proteins hnRNP A2/B1 and CUGBP1 that are involved in the new disease. RNA-binding proteins control the metabolism of mRNA. However, these RNA-binding proteins tend to bind to CGG repeats. When there are too many CGG repeats, too many molecules of these proteins are bound to the repeats, preventing them from fulfilling their normal function of controlling mRNA metabolism.
When Botas and his colleagues created a fly with too many CGG repeats, the fly developed the neurodegenerative disease. However, when they developed a fly that made more than the normal amount of the RNA-binding proteins, the disease was much less severe.
| Contact: Kimberlee Barbour kbarbour@bcm.edu 713-798-4712 Baylor College of Medicine Source:Eurekalert |