Target intelligence / Profile preview

Zinc finger NFX1-type containing 1 (ZNFX1)

Target
ZNFX1
Molecular classification
RNA helicase (superfamily 1, Upf1-like subfamily), Zinc finger protein, RNA-binding protein, Transcription factor (by domain similarity), Other
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Overview

**Zinc finger NFX1-type containing 1 (ZNFX1)** is a large, evolutionarily conserved protein encoded by the ZNFX1 gene, featuring a superfamily 1 (SF1) RNA helicase core, six NFX1-type zinc finger domains, an Armadillo-type fold (ARM) domain, a P-loop NTPase domain, a coiled-coil region, and a C-terminal RZ-type zinc finger[1][2][3]. Its main functions include the modulation of innate immune responses—regulating interferon signaling, mRNA stability (notably *Prkaa2*), suppression of excessive inflammation, and acting as a double-stranded RNA sensor in some contexts[1][2][3][4]. It is active in the cytoplasm and membrane, particularly in myeloid cells, and is essential for host defense against both viruses and bacteria (notably Mycobacterium tuberculosis)[1][2][3][5]. Deficiency of ZNFX1 results in a primary immunodeficiency with increased susceptibility to infections and severe systemic inflammation[1][5]. ZNFX1 is not currently a therapeutic target for any drugs, but its critical role in immune regulation makes it a subject of active biomedical research[1][3][5].

Other names
NFX1-type zinc finger-containing protein 1KIAA1404FLJ11277IMD91ZNFX1_HUMAN
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Mechanism of action

Not applicable (no direct drug modulators known as of current literature)

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Biological functions

Innate immune response modulatorDouble-stranded RNA sensor (in some cell types)mRNA stabilization and decay regulationSuppression of NLRP3 inflammasome activationAntiviral response mediatorRegulation of autophagy in macrophages
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Disease associations

Primary immunodeficiency (Immunodeficiency 91 and related hyperinflammation)Susceptibility to viral infectionsSusceptibility to mycobacterial infections (e.g., tuberculosis)Multisystem inflammatory disease (including liver, lung, kidney failure, seizures, and HLH)
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Safety considerations

Patients with ZNFX1 deficiency may develop life-threatening multisystem inflammatory diseases, severe infections, and altered responses to IFN signaling
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Biomarkers

ZNFX1 mutation as a biomarker for primary immunodeficiency and hyperinflammatory syndromes

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