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Fellowship Research Project Funded in 2014

Mechanisms associated with hyper-responsive PLT GPIbα cause alterations in PLT activation and function leading to a bleeding phenotype.

Dr. Harmanpreet Kaur
Queen’s University – Kingston, Ontario
One year funding. Start: April 30, 2014; to end September 2015.

GPIbα is a protein present on the membrane of platelets and is very important in clotting. It binds to another clotting protein called von Willebrand factor (VWF) and this binding is considered to be the first step of clotting at the site of injury. Hyper-responsive GPIbα characterizes the rare bleeding disorder platelet-type von Willebrand disease (PT-VWD) and results in excessive binding between GPIbα and VWF leading to subsequent removal from the circulation. Patients suffering from this disease have mild to severe bleeding problems which can be life threatening in case of surgery, pregnancy and childbirth if not treated properly.  Mechanisms that explain the bleeding condition are not fully understood and appear to be independent of VWF binding and clearance of VWF-platelet complex. We will look at various aspects of platelet activation and clot formation using a mouse model for PT-VWD. We will also study the effect of inhibiting hyper-response GPIbα on bleeding in these mice. This study is important because it could help us better understand this rare disease and to develop strategies to improve bleeding conditions in PT-VWD patients.