ZNFX1

ZNFX1 (Zinc Finger NFX1-Type Containing 1) is a Protein Coding gene. Gene Ontology (GO) annotations related to this gene include binding. An important paralog of this gene is AQR.

Full Name

ZNFX1

Function

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).

Biological Process

Biological Process activation of innate immune response Source:UniProtKB
Biological Process defense response to bacterium Source:UniProtKB1 Publication
Biological Process defense response to virus Source:UniProtKB1 Publication
Biological Process innate immune response Source:UniProtKB-KW
Biological Process negative regulation of viral genome replication Source:UniProtKB
Biological Process RNA-mediated heterochromatin formation Source:GO_Central1 Publication

Cellular Location

Mitochondrion outer membrane
Cytoplasm, Stress granule

Involvement in disease

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.