Mechanisms of Flow-regulated VWF-platelet Adhesion at Different Length Scales

Mechanisms of Flow-regulated VWF-platelet Adhesion at Different Length Scales

Year:
-
Grants:
Judith Graham Pool Postdoctoral Research Fellowship
Von Willebrand Disease
Author(s):
Klaus Bonazza

Klaus Bonazza received his Ph.D. in chemistry from Vienna University of Technology. He is currently a postdoctoral researcher at Boston Children's Hospital and appointed at Harvard Medical School, mentored by Dr. Timothy Springer. His field of interest is the ultra-large concatemeric protein von Willebrand factor (VWF), which accounts for the adaptability of hemostasis to different flow conditions in the blood vessels.

At moderate, physiological flow VWF has a packed, "bird nest's" shape whereas strong elongational flow conditions, occurring downstream of vascular restrictions or injuries, induce a transition to a threat-like, elongated state. On top of this overall unpacking, tensile forces, which are exerted on the chain and transmitted by its A1 domain, cause local conformational changes which activate binding of thrombocyte receptor Glycoprotein Ib (GPIbα) to initiate coagulation. With his JGP fellowship award, Dr. Bonazza will pioneer a new method to obtain structural insights into force dependent VWF unpacking, A1 deformation and GPIbα binding based on hydrogendeuterium exchange under elongational flow conditions.

Sabrina Farina

Improving the Quality of Life for the Aging Patient with Hemophilia

Year: 2018
Grants:
Social Work Excellence Fellowship
Author(s):
Sabrina Farina

As a social worker at Gulf States Hemophilia & Thrombophilia Treatment Center, I have the privilege of serving patients across their lifespan. I would like to initiate grass roots education about hemophilia in Houston, Texas, by offering educational programming to specialized health care professionals who work directly with the aging population in nursing homes and assisted living communities. This would include executive directors and administrators of these facilities as well as direct clinical staff.

Investigation of VWF as an Immunomodulator of the Immunogenic Response Towards FVIII

Investigation of VWF as an Immunomodulator of the Immunogenic Response Towards FVIII

Year: 2018
Grants:
Bridge Award
Von Willebrand Disease
Hemophilia A (Factor VIII/F8)
Inhibitors
Author(s):
Qizhen Shi

Dr. Shi is a Professor of Pediatric Hematology at the Medical College of Wisconsin and an Investigator of Blood Research Institute at the BloodCenter of Wisconsin. She earned her MD from Fujian Medical University in China in 1990 and her Ph.D. in 1998. Dr. Shi’s research focus is to formulate innovative therapeutic approaches for the treatment of hemophilia A, a genetic bleeding disorder caused by a lack of the critical blood clotting protein, factor VIII (FVIII). One of her research programs funded by the National Institutes of Health, National Heart, Lung, and Blood Institute, is to develop platelet-specific gene transfer strategies for the treatment of hemophilia A and hemophilia A with neutralizing antibodies. In the project supported by the NHF Bridge Grant, Dr. Shi will investigate the potential effect of the FVIII carrier protein, von Willebrand factor, on FVIII immune responses in hemophilia A. Dr. Shi expects that results from her studies will aid the design of more effective protocols to prevent FVIII immune responses and to induce FVIII immune tolerance in patients with HA.

Impact of hemophilia on employment - Insights from the PROBE Study

Impact of hemophilia on employment - Insights from the PROBE Study

AWARDED/PRESENTED: 2018
GRANT/PROGRAM:
Bleeding Disorders Conference
Quality of Life/Outcomes Research
RESEARCHERS:
Alexandra Pastarnak, Brian O'Mahony, Chatree Chai-Adisaksopha, David Page, Randall Curtis, Alfonso Iorio, Jeff Stonebraker, Michael Nichol, Mark Skinner, Declan Noone