Hemophagocytic lymphohistiocytosis–like hyperinflammation due to a de novo mutation in DPP9

BACKGROUND: Genetic defects in components of inflammasomes can cause autoinflammation. Biallelic loss-of-function mutations in dipeptidyl peptidase 9 (DPP9), a negative regulator of the NLRP1 and CARD8 inflammasomes, have recently been shown to cause an inborn error of immunity characterized by panc...

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Bibliographic Details
Main Authors: Wolf, Christine, Fischer, Hannah, Kühl, Jörn-Sven, Koss, Sarah, Jamra, Rami Abou, Starke, Sven, Schultz, Jurek, Ehl, Stephan, Neumann, Katrin, Schuetz, Catharina, Huber, Robert, Hornung, Veit, Lee-Kirsch, Min Ae
Format: Text
Language:English
Published: 2023
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Online Access:https://pmc.ncbi.nlm.nih.gov/articles/PMC7615848/
https://pubmed.ncbi.nlm.nih.gov/37544411
http://dx.doi.org/10.1016/j.jaci.2023.07.013
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