5716998999ENSG00000124201ENSMUSG00000039501Q9P2E3Q8R151NM_021035NM_001033196NM_001291162NP_066363NP_001028368NP_001278091Zinc finger NFX1-type containing 1 is a protein that in humans is encoded by the ZNFX1 gene.

[5] ZNFX1 deficiency in humans is associated with severe multisystem inflammatory disease.

[6] Affected individuals may develop liver, lung or kidney failure, seizures and hemophagocytic lymphohistiocytosis (HLH) upon viral infections.

Intermittent monocytosis is a hallmark laboratory finding in ZNFX1 deficiency.

The disorder is thought to result from alterations in the half-life of the mRNA of interferon-stimulated genes (ISG) and is also associated with poorer clearance of viral infections in monocytes.