Grant: Operating Grant

Defects in cytoskeletal proteins have a primary role in many neurodegenerative diseases. In working towards therapies for these diseases, it is important to understand the basic context in which the conditions occur. Implicit in this is an understanding of the basic architectural makeup of cells and the molecular determinants that are required in establishing a normal framework. Our work on dystonin cytoskeletal proteins show that they are fundamental in facilitating communication from the nucleus to the plasma membrane by ensuring the stability of the cellular scaffold. The work proposed will shed light on the role of this protein in intracellular trafficking and organelle organization. Since cytoskeletal defects are central features of many neurodegenerative disorders, elucidation of the exact role of dystonin in neurons should not only enhance our understanding of the versatile nature of these large modular proteins, but should help provide fundamental insight into the aetiology of these disorders.