IFI44L

Chr 1

interferon induced protein 44 like

Also known as: C1orf29, GS3686, TLDC5B

This interferon-stimulated gene encodes a protein that provides antiviral and antibacterial activity by promoting macrophage differentiation, facilitating inflammatory cytokine secretion, and controlling viral replication, while also serving as a feedback regulator of interferon responses. Mutations in IFI44L cause autosomal recessive immunodeficiency, with patients presenting with severe, recurrent infections affecting multiple organ systems. The gene shows very low constraint against loss-of-function variants, consistent with a recessive inheritance pattern.

Summary from RefSeq, UniProt
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2
Active trials
51
Pubs (1 yr)
15
P/LP submissions
0%
P/LP missense
1.52
LOEUF
DN
Mechanism· predicted
Clinical SummaryIFI44L
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
15 unique Pathogenic / Likely Pathogenic· 77 VUS of 119 total submissions
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.52LOEUF
pLI 0.000
Z-score -0.10
OE 1.03 (0.711.52)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.21Z-score
OE missense 1.22 (1.111.34)
299 obs / 245.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.03 (0.711.52)
00.351.4
Missense OE1.22 (1.111.34)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 18 / 17.6Missense obs/exp: 299 / 245.7Syn Z: 0.16
DN
0.6161th %ile
GOF
0.5465th %ile
LOF
0.2580th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

119 submitted variants in ClinVar

Classification Summary

Pathogenic14
Likely Pathogenic1
VUS77
Likely Benign7
Benign3
14
Pathogenic
1
Likely Pathogenic
77
VUS
7
Likely Benign
3
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
14
0
14
Likely Pathogenic
0
0
1
0
1
VUS
1
65
11
0
77
Likely Benign
0
5
1
1
7
Benign
0
2
0
1
3
Total172272102

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

IFI44L · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Literature
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