Lisa Westerberg

Project summary

Immunodeficiency disorders

Our immune system never rests. Immune cells constantly circulate throughout the body, enabling them to rapidly detect and eliminate foreign infectious agents. Considering the enormous response that immune cells can mount to eliminate such threats raises two fundamental questions: Why does our immune system not attack our own tissues, and how does it know when to stop immune-cell proliferation?

 

The project studies immunodeficiency disorders in order to improve our understanding of the immune system. In healthy individuals, the essential mechanisms that regulate immune responses work extremely well – allowing us, for example, to recover from a common cold. However, a group of life-threatening conditions known collectively as primary immunodeficiency disorders, which affect newborns and young children, can lead to uncontrolled regulation of patients’ immune cells.

 

Two of these primary immunodeficiency disorders are caused by mutations affecting the protein WASP, which is important for immune-cell movement, communication with other cells and cell division. While the immune systems of affected children fail to activate normally in response to infections such as the common cold, the children are also at high risk of developing immune reactions against their own tissues and cancers of the blood cells.

 

The project uses experimental models of immunodeficiency disorders caused by WASP mutations to determine which components of the immune system fail to function normally and subsequently to elucidate the underlying mechanisms responsible for this altered regulation.

 

The aim of the studies is to increase our understanding of how a dysregulated immune system leads to the development of immunodeficiency disorders in children and to develop new treatment approaches using emerging research technologies. To achieve this, the research group brings together students and researchers from a range of disciplines, from basic scientists to physicians. Within five years, we hope to understand how mutations in WASP cause immunodeficiency and, in doing so, gain a better understanding of how the immune system functions in both health and disease. Another ambition is to identify new treatment approaches with the potential for future use in patients.