ZNFX1

RNA-binding protein that initiates the antiviral response and is required to restrict the replication of RNA viruses (PubMed:33872655). Acts as a double-stranded RNA (dsRNA) sensor that recognizes viral RNA and then interacts with MAVS to initiate the type I interferon response (By similarity). Also required for immunity against some bacteria, such as mycobacteria (PubMed:33876776).

Immunodeficiency 91 and hyperinflammation

IMD91

An autosomal recessive disorder characterized by immunodeficiency, recurrent infections, and hyperinflammation with systemic involvement. Most patients eventually develop hepatic or renal failure, may have compromised neurologic function, lymphadenopathy or hepatosplenomegaly. Early death often occurs due to multiorgan failure.

None

The disease is caused by variants affecting the gene represented in this entry.

Belongs to the ZNFX1 family.

Widely expressed.

• Mitochondrion outer membrane
• Cytoplasm
• Stress granule

KIAA1404, ZNFX1, NFX1-type zinc finger-containing protein 1

• entrezGene:57169
• swissprot:Q9P2E3

Proteins

220227Da