Abstract:

Although all cells in an organism contain the same genetic information, spatial and temporal control of gene expression dictate cell physiology and function.  Much of this complex regulation is accomplished through post-transcriptional mechanisms via evolutionarily conserved RNA binding. These proteins are critical for many steps in gene expression, a point which is highlighted by the growing number of links between such RNA binding proteins and human disease. Curiously, although these RNA binding proteins are conserved and critical for gene expression, mutations in the genes that encode them often cause tissue-specific disease. Thus, the challenge is two-fold, first to define the mechanistic role of these proteins in ensuring proper gene expression and second, to understand the biological context of that function in the tissue impacted in disease. This seminar will focus on our recent discovery of an RNA binding protein, ZC3H14, that is lost in an inherited neurological disease. Our studies employ integrated approaches in model systems from yeast and flies to mice with the goal of uncovering the critical biological process(es) where ZC3H14 is required for proper neuronal function.