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Article Dans Une Revue Molecular Immunology Année : 2017

Hereditary C1 inhibitor deficiency is associated with high spontaneous amidase activity

Résumé

function and antigenic C4 level) and contact phase activation (via amidase activity). Bradykinin is respon-sible for angioedema attacks and is produced from contact phase activation secondary to failed C1Inhcontrol.Objective: We aimed to compare the diagnostic performances of spontaneous amidase activity and anti-genic C4 level in C1Inh hereditary angioedema (C1Inh-HAE) patients.Methods: Samples from 185 C1Inh-HAE patients (81 men, 104 women; confirmed by SERPING1 genemutations) and from 99 blood donors (50 men, 49 women) were tested for C1Inh function, antigenic C4level and spontaneous amidase activity.Results: In the C1Inh-HAE group, antigenic C4 level was decreased (n = 135) and amidase activity wasincreased (n = 181). Receiver operating characteristic analyses showed higher diagnostic performancevalues for the spontaneous amidase assay compared to those of antigenic C4.Conclusion: The spontaneous amidase activity assay should replace antigenic C4 level testing and shouldbe tested alongside the C1Inh function for both AE screening and follow up of HAE patients.
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Dates et versions

hal-04540454 , version 1 (22-04-2024)

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Citer

Delphine Charignon, Arije Ghannam, Denise Ponard, Christian Drouet. Hereditary C1 inhibitor deficiency is associated with high spontaneous amidase activity. Molecular Immunology, 2017, 85, pp.120-122. ⟨10.1016/j.molimm.2017.01.028⟩. ⟨hal-04540454⟩

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