The quality control of mislocalised membrane and secretory proteins


  • Prof Stephen High

    University of Manchester

Project summary

Proteins are essential to life, providing important building blocks and performing complex roles that maintain health and fight disease. Making proteins is complicated, so we have systems to recognise and remove any misfolded proteins that arise when things go wrong. The ability to control the quality of our proteins is important, since faulty proteins often clump together into potentially dangerous aggregates that are linked to diseases like Alzheimer’s and Huntington’s. Rogue membrane and secretory proteins that reach the wrong destination in our cells, collectively termed muscle LIM proteins (MLPs), also have a strong tendency to aggregate. However, a specialised quality control process recognises and removes these MLPs thereby avoiding potential problems.

We have identified important cellular factors that deal with MLPs by controlling their aggregation and fast-tracking their destruction. It is now vital to understand exactly how these different components work together to deal with MLPs so effectively.

By discovering how MLP quality control works and learning how cells cope when it does not, we will find out why cells can deal with certain kinds of misfolded proteins so effectively and discover how important efficient MLP quality control is for keeping our cells healthy.