In:
Science Immunology, American Association for the Advancement of Science (AAAS), Vol. 7, No. 75 ( 2022-09-30)
Abstract:
The NLRP1 inflammasome is a protein complex crucial for various immune responses; thus, dysregulation of it can lead to severe immune disorders. Here, Harapas et al . looked at four children with dipeptidyl peptidase 9 (DPP9) loss-of-function mutations, showing that these patients had various immune disorders and spontaneous activation of the NLRP1 inflammasome in their isolated keratinocytes. Modeling these patients with a mouse model with a catalytically inactive DPP9 mutation, they found that neonate lethality could be rescued by also knocking out NLRP1. In a zebrafish model, knockout of asc, a molecule involved in NLRP1 inflammasome formation, also rescued the survival of dpp9 knockout zebrafish. Thus, DPP9 mutations seem to result in immune disorders due to NLRP1 inflammasome overactivation.
Type of Medium:
Online Resource
ISSN:
2470-9468
DOI:
10.1126/sciimmunol.abi4611
Language:
English
Publisher:
American Association for the Advancement of Science (AAAS)
Publication Date:
2022
detail.hit.zdb_id:
2862556-0
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