CTLA4-Ig effectively controls immune activation and inflammatory disease in a novel murine model of leaky SCID.
- Stéphanie Humblet-Baron,
- Susann Schönefeldt,
- Josselyn Garcia-Perez,
- Frédéric Baron,
- Emanuela Pasciuto,
- Adrian Liston
J Allergy Clin Immunol 2017 Feb 6. Epub 2017 Feb 6.
Translational Immunology Laboratory, VIB, Leuven, Belgium; KU Leuven - University of Leuven, Department of Microbiology and Immunology, Leuven, Belgium. Electronic address:
Background: Severe combined immunodeficiency (SCID) can be caused by loss-of-function mutations in genes involved in the DNA recombination machinery, such as RAG1, RAG2 or DCLRE1C. Defective DNA recombination causes a developmental block in T cells and B cells, resulting in high susceptibility to infections. Hypomorphic mutations in the same genes can also give a partial loss of T cells, in a spectrum including leaky SCID (LS) and Omenn syndrome (OS). Read More