In eucaryotic cells, destined for the cell surface or for the external milieu are first translocated into the endoplasmic reticulum (ER) where initial protein folding and modifications occur that are essential for the protein to attain its appropriate functional conformation. Recently, there have been significant breakthroughs in our understanding of the cellular machinery that direct protein folding, that control the post- translational modification of nascent polypeptides, and that recognize aberrant proteins and target them for degradation. These processes are necessary to ensure fidelity of the final protein product. A number of ER molecular chaperones have been identified that interact transiently with folding intermediates and their roles in facilitating and monitoring these processes during protein biosynthesis are rapidly being determined. This conference will focus on the combinations of genetic, biochemical, and cell biological approaches that are currently being used to elucidate the initial steps in the biosynthesis of proteins in the secretory pathway. Because many diseases can be attributed to aberrations in these processes, several sessions will be devoted to abnormal protein folding and the cellular mechanisms for monitoring and responding to incorrectly folded or assembled proteins. To more directly address the role of abnormal protein folding leading to disease, there will be talks dealing with specific proteins that when mutated result in incorrectly folded proteins which are at the heart of the disease process. It is our intention that will meeting will combine basic researchers who are probing the mechanisms of protein folding and chaperone interactions with more applied or clinical researchers who are identifying the underlying cause of various diseases at the protein level. We feel that this interaction between the groups will provide applied researchers with an understanding of what is currently known about how protein folding and transport through the secretory pathway are controlled and will provide basic researcher with biologically relevant model systems to study these processes. We anticipate that the interaction of these two groups of researchers will lead to new collaborations and better understanding of certain disease states and may ultimately lead to new approaches for intervention and treatment.
