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Murine Models of Familial Cytokine Storm Syndromes.
- Source :
-
Advances in experimental medicine and biology [Adv Exp Med Biol] 2024; Vol. 1448, pp. 481-496. - Publication Year :
- 2024
-
Abstract
- Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disease caused by mutations in effectors and regulators of cytotoxicity in cytotoxic T cells (CTL) and natural killer (NK) cells. The complexity of the immune system means that in vivo models are needed to efficiently study diseases like HLH. Mice with defects in the genes known to cause primary HLH (pHLH) are available. However, these mice only develop the characteristic features of HLH after the induction of an immune response (typically through infection with lymphocytic choriomeningitis virus). Nevertheless, murine models have been invaluable for understanding the mechanisms that lead to HLH. For example, the cytotoxic machinery (e.g., the transport of cytotoxic vesicles and the release of granzymes and perforin after membrane fusion) was first characterized in the mouse. Experiments in murine models of pHLH have emphasized the importance of cytotoxic cells, antigen-presenting cells (APC), and cytokines in hyperinflammatory positive feedback loops (e.g., cytokine storms). This knowledge has facilitated the development of treatments for human HLH, some of which are now being tested in the clinic.<br /> (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)
- Subjects :
- Animals
Mice
Humans
Cytokines metabolism
Cytokines genetics
T-Lymphocytes, Cytotoxic immunology
Killer Cells, Natural immunology
Disease Models, Animal
Lymphohistiocytosis, Hemophagocytic immunology
Lymphohistiocytosis, Hemophagocytic genetics
Cytokine Release Syndrome immunology
Cytokine Release Syndrome genetics
Cytokine Release Syndrome pathology
Subjects
Details
- Language :
- English
- ISSN :
- 0065-2598
- Volume :
- 1448
- Database :
- MEDLINE
- Journal :
- Advances in experimental medicine and biology
- Publication Type :
- Academic Journal
- Accession number :
- 39117835
- Full Text :
- https://doi.org/10.1007/978-3-031-59815-9_33