Phenotypical and Functional Characterization of Neutrophils in Two Pyrin-Associated Auto-inflammatory Diseases | Zendy

Bert MalengierDevlies | Zendy; Mieke Metzemaekers | Zendy; Mieke Gouwy | Zendy; Erika Van Nieuwenhove | Zendy; Albrecht Betrains | Zendy; Maaike Cockx | Zendy; Lotte Vanbrabant | Zendy; Noëmie Pörtner | Zendy; Jurgen Vercauteren | Zendy; Lien De Somer | Zendy; Sofie Struyf | Zendy; Steven Vanderschueren | Zendy; Ellen De Langhe | Zendy; Paul Proost | Zendy; Patrick Matthys | Zendy; Carine Wouters | Zendy

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Phenotypical and Functional Characterization of Neutrophils in Two Pyrin-Associated Auto-inflammatory Diseases

Author(s) -

Bert MalengierDevlies,

Mieke Metzemaekers,

Mieke Gouwy,

Erika Van Nieuwenhove,

Albrecht Betrains,

Maaike Cockx,

Lotte Vanbrabant,

Noëmie Pörtner,

Jurgen Vercauteren,

Lien De Somer,

Sofie Struyf,

Steven Vanderschueren,

Ellen De Langhe,

Paul Proost,

Patrick Matthys,

Carine Wouters

Publication year - 2021

Publication title -

journal of clinical immunology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.739

H-Index - 96

eISSN - 1573-2592

pISSN - 0271-9142

DOI - 10.1007/s10875-021-01008-4

Subject(s) - pyrin domain , mefv , familial mediterranean fever , s100a9 , phenotype , immunology , myeloperoxidase , degranulation , s100a8 , chemotaxis , inflammasome , biology , inflammation , tlr4 , downregulation and upregulation , mmp9 , receptor , gene , medicine , genetics , pathology , gene mutation , mutation , disease

Familial Mediterranean Fever (FMF) and Pyrin-Associated Autoinflammation with Neutrophilic Dermatosis (PAAND) are clinically distinct autoinflammatory disorders caused by mutations in the pyrin-encoding gene MEFV. We investigated the transcriptional, phenotypical, and functional characteristics of patient neutrophils to explore their potential role in FMF and PAAND pathophysiology.

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