Table 1.

Common characteristics of IE-IRFs

GeneIRF1IRF3IRF4IRF6IRF7IRF8IRF9
Inheritance patternARAD (HSE)ADAD (T95R)AD (F359L)AD (R98W)AR (V405Gfs
Ter127)		AD (VWS)AD (PPS)ARADARADAR
MechanismLOFHIHIMMMMHIUPDHIDNLOF?LOFDNLOF
Bacterial susceptibility ✔ ​ ​ ✔ ✔ ✔ ? ​ ​ ​ ​ ✔ ✔ ​ 
Viral susceptibility ​ ✔ ✔ ✔ ✔ ​ ✔ ​ ​ ✔ ✔ ✔ ✔ ✔ 
Known pathogen range Weakly virulent mycobacteria
+ Histoplasma capsulatum 		HSV Respiratory viral infections: Severe SARS-CoV-2 and influenza P. jirovecii, vaccine strain BCG, M. bovis, CMV, EBV, … (see Table 2) CMV, HSV, VZV, oral mycosis, G. lamblia, onychomycosis, … (see Table 2) Tw Rotavirus, C. albicans, HSV, respiratory infections (unknown cause) No known infection susceptibility No known infection susceptibility Respiratory viral infections: severe SARS-CoV-2 and influenza
+ RSV, TBE virus, and adenovirus 		Respiratory viral infections: severe SARS-CoV-2 and influenza Weakly virulent mycobacteria Respiratory viral infections: severe SARS-CoV-2 and influenza
+ eight other viruses (see Table 2) 
+ EBV, influenza, C. albicans, rhinovirus, HPV, CMV, and mycoplasma + EBV, HPV, recurrent Clostridium difficile 
Cellular abnormalities CD4+ T cells ↑↓ ​ ​ ↓ ↑↓ ​ ↑↓ ​ ​ ​ ​ ↓ ↑ ↓ 
CD8+ T cells ↑↓ ​ ​ ​ ↑↓ ​ ↓ ​ ​ ​ ​ ↓ ​ ​ 
NK cells ↓↓ ​ ​ ​ ​ ​ ​ ​ ​ ​ ​ ↓ ​ ↓ 
B cells ↑ ​ ​ ↓↓ ↓ ​ ↓ ​ ​ ​ ​ ↓ ↓ ↓ 
DCs ↓ ​ ​ ​ ​ ​ ↑↓ ​ ​ ​ ​ ↓↓/↓↓↓ ↓ ​ 
Monocytes ​ ​ ​ ​ ​ ​ ↑↓ ​ ​ ​ ​ ↓↓/↓↓↓ ↑ ​ 
IFN impairment IFN-γ IFN-α/β IFN-α/β IFN-γ ​ IFN-γ IFN-γ ​ ​ IFN-α/β/λ1 IFN-α/β IFN-γ IFN-α IFN-α 
Gene expression defect ↓ ↓ ↓ ↑↓ ↑↓ ↓ ↓ Likely ↓ ↓ ↓ ↓ ↓ ↓ 
Other defects Reduced innate lymphoid precursors ​ ​ Hypogammaglobulinemia Hypogammaglobulinemia, premature hair graying ​ Agammaglobulinemia, hypereosinophilia, and hemophagocytosis Lip pits and CLP Lip pits, CLP, popliteal webbing, syndactyly, hypodontia, and deformities of the limbs and genitals Impaired viral control in fibroblasts and pulmonary epithelia Impaired IAV control in macrophages Increased hypofunctional granulocytes. Intracerebral calcification and developmental delay Mild neutrophilia Impaired IAV control in fibroblasts, hypogammaglobulinemia 

HI, haploinsufficiency; DN, dominant negative; MM, multimorphic; HSE, herpes simplex encephalitis; LOF, loss of function; TBE, tick-borne encephalitis; cellular abnormalities, abnormal count/function; IFN impairment, abnormal production/response; ↑, elevated; ↓, reduced; ↑↓, mixed effect or variable by genotype. See Table 2 for a detailed characterization of each IE-IRF.

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