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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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AZI2
5-azacytidine induced 2
Chromosome 3 · 3p24.1
NCBI Gene: 64343Ensembl: ENSG00000163512.15HGNC: HGNC:24002UniProt: Q9H6S1
49PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingcytoplasmdefense response to virustype I interferon-mediated signaling pathwayneurodegenerative diseasesmoking initiationtype 2 diabetes mellitusParkinson disease
✦AI Summary

AZI2 (5-azacytidine induced 2) is an adapter protein that plays critical roles in antiviral immunity, cell death prevention, and selective autophagy. Functionally, AZI2 binds TBK1 and IKKε, facilitating TBK1 oligomerization and enhancing phosphorylation of NF-κB p65 subunit RELA to promote NF-κB activation 12. AZI2 participates in type I interferon production via the IFNB promoter through TICAM1-dependent signaling 3. Mechanistically, AZI2 operates through distinct recruitment kinetics in TNF signaling: it is initially recruited via deubiquitinase A20, working alongside TANK adapter to sustain TBK1 activation at the TNF receptor complex, where TBK1 phosphorylates RIPK1 to prevent cell death 4. AZI2 also regulates NDP52-driven mitochondrial autophagy, with its S318 phosphorylation by TBK1 modulating mitophagy efficiency 5. TBK1 sustains AZI2 abundance and phosphorylation state, critical for maintaining autophagy-related protein homeostasis in neurons 6. Clinically, AZI2 positively regulates type I interferon responses in influenza-induced pediatric pneumonia; AZI2-deficient mice show deteriorated pathology and reduced survival 7. In cancer immunotherapy, AZI2-mediated TBK1 activation downstream of autophagy disruption enhances pro-inflammatory chemokine expression and CD8+ T-cell tumor infiltration, offering a sensitization strategy for immunotherapy-resistant breast cancers 8.

Sources cited
1
AZI2 binds TBK1, activates it, facilitates oligomerization, and enhances NF-κB p65 phosphorylation
PMID: 14560022
2
AZI2 role in TBK1 activation, NF-κB signaling, and antiviral innate immunity
PMID: 21931631
3
AZI2 participates in IFNB promoter activation via TICAM1
PMID: 15611223
4
AZI2 recruits TBK1 via A20 to prevent TNF-induced cell death and autoinflammation
PMID: 39562788
5
AZI2 regulates NDP52-driven mitochondrial autophagy through TBK1-mediated phosphorylation at S318
PMID: 39276928
6
TBK1 sustains AZI2 abundance and phosphorylation in autophagy networks critical for neuronal protein homeostasis
PMID: 41171761
7
AZI2 positively regulates type I interferon production in influenza-induced pneumonia; AZI2 deficiency worsens disease
PMID: 35595469
8
AZI2-mediated TBK1 activation enhances pro-inflammatory chemokine expression and CD8+ T-cell infiltration in breast cancer
PMID: 37733921
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.44Moderate
smoking initiationOpen Targets
0.26Weak
type 2 diabetes mellitusOpen Targets
0.23Weak
Parkinson diseaseOpen Targets
0.22Weak
DNA methylationOpen Targets
0.21Weak
AnisometropiaOpen Targets
0.19Weak
COVID-19Open Targets
0.13Weak
non-small cell lung carcinomaOpen Targets
0.07Suggestive
colorectal cancerOpen Targets
0.07Suggestive
nasopharyngeal carcinomaOpen Targets
0.05Suggestive
multiple sclerosisOpen Targets
0.05Suggestive
systemic lupus erythematosusOpen Targets
0.04Suggestive
autoimmune diseaseOpen Targets
0.03Suggestive
AphasiaOpen Targets
0.03Suggestive
immunodeficiency 89 and autoimmunityOpen Targets
0.03Suggestive
clostridium difficile infectionOpen Targets
0.03Suggestive
hyper-IgE recurrent infection syndrome 5, autosomal recessiveOpen Targets
0.03Suggestive
connective tissue diseaseOpen Targets
0.03Suggestive
primary thrombocytopeniaOpen Targets
0.03Suggestive
hematopoietic and lymphoid cell neoplasmOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TAX1BP1Protein interaction100%CALCOCO2Protein interaction100%TRAF3Protein interaction100%TNFProtein interaction98%IKBKGProtein interaction98%IKBKEProtein interaction98%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
85%
Brain
82%
Heart
77%
Ovary
58%
Liver
45%
Gene Interaction Network
Click a node to explore
AZI2TAX1BP1CALCOCO2TRAF3TNFIKBKGIKBKE
PROTEIN STRUCTURE
Preparing viewer…
PDB5EP6 · 1.45 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.69LoF Tolerant
pLIⓘ
0.09Tolerant
Observed/Expected LoF0.44 [0.30–0.69]
RankingsWhere AZI2 stands among ~20K protein-coding genes
  • #8,913of 20,598
    Most Researched49
  • #5,135of 17,882
    Most Constrained (LOEUF)0.69
Genes detectedAZI2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
TBK1 and IKKε prevent TNF-induced cell death by RIPK1 phosphorylation.
PMID: 30420664
Nat Cell Biol · 2018
1.00
2
TBK1-associated adapters TANK and AZI2 protect mice against TNF-induced cell death and severe autoinflammatory diseases.
PMID: 39562788
Nat Commun · 2024
0.90
3
TBK1 adaptor AZI2/NAP1 regulates NDP52-driven mitochondrial autophagy.
PMID: 39276928
J Biol Chem · 2024
0.80
4
Phospho-proteome profiling in human neurons reveals targets of TBK1 in ALS/FTD-associated autophagy networks.
PMID: 41171761
Cell Rep · 2025
0.70
5
AZI2 positively regulates the induction of type I interferon in influenza-trigger pediatric pneumonia.
PMID: 35595469
Pathog Dis · 2022
0.60