Interleukin-18 binding protein deficiency

Definition:

Interleukin-18 binding protein deficiency is caused by inborn errors in IL18BP, which encodes IL-18 binding protein (IL-18BP), resulting in fulminant viral hepatitis (FVH) by unleashing IL-18. IL-18BP is the naturally occurring antagonist of IL-18.

References:

[1]. Julia E Rood, et al. Inherited Autoinflammatory Syndromes. Annu Rev Pathol. 2022 Jan 24;17:227-249. [Content Brief]

[2]. Serkan Belkaya, et al. Inherited IL-18BP deficiency in human fulminant viral hepatitis. J Exp Med. 2019 Aug 5;216(8):1777-1790. [Content Brief]

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