Immune Defects
Haemophagocytic lymphohistiocytosis (HLH) is an often fatal disease of the immune system which develops by various genetic defects of the cytotoxic protein perforin or membrane trafficking-associated proteins.
The functional consequence is an inefficient immunological response in viral infections and an encroaching proliferation of macrophages within the scope of a "cytokine-storm". HLH on the basis of germline mutations can ultimately only be heated by a transplantation of blood stem cells.
Our research concentrates on the identifcation of additional genes, whose mutation initiates the phenotype of HLH. The impact of the vesicle-associated transport protein Syntaxin 11 on HLH is examined by gene-knock-out strategy in a mouse model. These studies shall provide an essential understanding about the cytotoxic immune defense against viral infections.