Abstract
Caspase-1, belonging to the family of cystein proteases, is well-known for its involvement in IL-1β and IL-18 maturation. Although, caspase-1 is one of the best described caspase-family-members, open questions concerning autoinflammatory diseases, caused by procaspase-1 variants (e.g. ICE-Fever), remain. ICE-Fever patients suffer from chronic febrile episodes, although procaspase-1 variants show reduced enzymatic activity leading to limited IL-1β secretion. The paradox of reduced IL-1β secretion but increased inflammation led to the hypothesis, that CASP1-variants enhance alternative signaling pathways. However, the role of procaspase-1 variants and their interaction partners during IL-1β maturation or NF-κB activation is still poorly understood.
Highlights
Caspase-1, belonging to the family of cystein proteases, is well-known for its involvement in IL-1b and IL-18 maturation
ICE-Fever patients suffer from chronic febrile episodes, procaspase-1 variants show reduced enzymatic activity leading to limited IL-1b secretion
The paradox of reduced IL-1b secretion but increased inflammation led to the hypothesis, that CASP1-variants enhance alternative signaling pathways
Summary
Caspase-1, belonging to the family of cystein proteases, is well-known for its involvement in IL-1b and IL-18 maturation. Caspase-1 is one of the best described caspase-family-members, open questions concerning autoinflammatory diseases, caused by procaspase-1 variants (e.g. ICE-Fever), remain. ICE-Fever patients suffer from chronic febrile episodes, procaspase-1 variants show reduced enzymatic activity leading to limited IL-1b secretion. The paradox of reduced IL-1b secretion but increased inflammation led to the hypothesis, that CASP1-variants enhance alternative signaling pathways. The role of procaspase-1 variants and their interaction partners during IL-1b maturation or NF-B activation is still poorly understood
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