… germline or somatic NLRC4 mutations. Herein, we summarize the growing body of literature
… NLRC4 mutation-associated autoinflammatory diseases, the NLRC4 inflammasomopathies. …

… set the NLRC4 inflammasomopathy apart from the PYRIN and NLRP3 inflammasomopathies,
… Prior to the description of NLRC4-related disease, MAS had very rarely been associated …

… (CAPS) and NLRC4-associated autoinflammatory disease are 2 inflammasomopathies
that are a consequence of gain-of-function mutations in the NLRP3 and NLRC4 genes, …

… Clinical differences in patients with NLRP3, pyrin, and NLRP1 inflammasomopathies, and
… with gain-of function mutations in NLRC4 led to the screening and the characterization of …

… germline NLRC4 mutations.5 14 These choices may also be effective for our patient with
NLRC4 … of using disulfiram to treat patients with NLRC4-associated inflammasomopathies. …

… NLRC4 … NLRC4 512 C> T (p.Ser171Phe) de novo mutation predicated to cause a dominant,
gain-of-function mutation resulting in a constitutively active protein. The assembly of NLRC4-…

… of inflammasomes (also known as inflammasomopathies) results in severe autoinflammation…
and Canna, NLRC4-related diseases differ from other inflammasomopathies largely in three …

… to other inflammasomopathies that are mainly driven by increased release of IL-1β, IL-18
was suggested to be the main cytokine responsible for the clinical manifestations of NLRC4-…

… Herein, we describe two new patients with NLRC4 mutations. The … NLRC4 mutations, on
exon 4, within the nucleotide-binding protein domain (NBD). After a systematic review of NLRC4 …

Inflammasomes are macromolecular complexes involved in the host response to external and
endogenous danger signals. Inflammasome-mediated sterile inflammation plays a central …