Studying storage granules to understand bleeding disorders

A novel high-throughput morphometrical analysis of blood outgrowth endothelial cells to determine the role of Weibel-Palade bodies in a variety of bleeding disorders

Endothelial cells that line the blood vessel contain cigar shaped storage granules called Weibel-Palade Bodies (WPBs), which release factors involved in blood clotting and inflammation. WPBs are malformed and do not function properly in Von Willebrand disease, a condition that causes abnormal clotting, and it is thought that they may be abnormal in other bleeding disorders. Researchers usually study WPBs from donated umbilical cords, but the BHF has now awarded a grant to Professor Daniel Cutler from University College London to study a new source of WPBs - endothelial cells that have been grown in the laboratory from patients’ blood. His team will study cells and WPBs from patients with bleeding disorders under the microscope, and analyse images using a computer that spots differences between healthy and diseased WPBs. The researchers hope this project will significantly improve our understanding of WPBs and bleeding diseases. It may reveal whether people with bleeding disorders have defective WPBs, which could reveal new avenues to treat people with these conditions.