TLR4

Chr 9

toll like receptor 4

Also known as: ARMD10, CD284, TLR-4, TOLL

The protein encoded by this gene is a member of the Toll-like receptor (TLR) family which plays a fundamental role in pathogen recognition and activation of innate immunity. TLRs are highly conserved from Drosophila to humans and share structural and functional similarities. They recognize pathogen-associated molecular patterns that are expressed on infectious agents, and mediate the production of cytokines necessary for the development of effective immunity. The various TLRs exhibit different patterns of expression. In silico studies have found a particularly strong binding of surface TLR4 with the spike protein of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of Coronavirus disease-2019 (COVID-19). This receptor has also been implicated in signal transduction events induced by lipopolysaccharide (LPS) found in most gram-negative bacteria. Mutations in this gene have been associated with differences in LPS responsiveness, and with susceptibility to age-related macular degeneration. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Aug 2020]

9
Active trials
25
Pathogenic / LP
163
ClinVar variants
7
Pubs (1 yr)
0.6
Missense Z
0.99
LOEUF
Clinical SummaryTLR4
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
25 Pathogenic / Likely Pathogenic· 106 VUS of 163 total submissions
💊
Clinical Trials
9 active or recruiting trials — potential therapeutic options may be available
📖
GeneReview available — TLR4
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?LOEUF (Loss-of-function Observed/Expected Upper bound Fraction) is the upper bound of the 90% CI for LoF OE — the preferred gnomAD v4 metric. Lower = more intolerant to LoF. LOEUF < 0.35 = highly constrained.
0.99LOEUF
pLI 0.000
Z-score 1.59
OE 0.66 (0.450.99)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?Missense Z-score: standard deviations fewer missense variants observed vs. expected. Z > 3.09 (p < 0.001) = gene does not tolerate missense variation. OE missense < 0.6 is also considered constrained.
0.65Z-score
OE missense 0.91 (0.840.99)
379 obs / 416.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?Shaded band = 90% confidence interval. Vertical tick = point estimate. Grey threshold line = gnomAD constraint cutoff for that variant class.
LoF OE?Ratio of observed to expected LoF variants. Upper CI bound (LOEUF) ≤ 0.35 = strong LoF constraint signal.0.66 (0.450.99)
00.351.4
Missense OE?Ratio of observed to expected missense variants. OE ≤ 0.6 = fewer missense variants than expected by chance.0.91 (0.840.99)
00.61.4
Synonymous OE?Control metric — synonymous variants are largely neutral and expected near OE = 1.0. Significant deviation may indicate annotation issues.0.96
01.21.6
LoF obs/exp: 17 / 25.7Missense obs/exp: 379 / 416.1Syn Z: 0.38
DN
0.78top 25%
GOF
0.78top 25%
LOF
0.1993th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction 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

163 submitted variants in ClinVar

Classification Summary

Pathogenic22
Likely Pathogenic3
VUS106
Likely Benign24
Benign7
Conflicting1
22
Pathogenic
3
Likely Pathogenic
106
VUS
24
Likely Benign
7
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
22
0
22
Likely Pathogenic
0
0
3
0
3
VUS
0
84
22
0
106
Likely Benign
0
13
3
8
24
Benign
0
4
1
2
7
Conflicting
1
Total01015110163

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

View in ClinVar →

Protein Context — Lollipop Plot

TLR4 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Chronic PainLiving Kidney DonorQuadratus Lumborum Block

The Effect of Quadratus Lumborum (QL) Block on the Incidence of Chronic Neuropathic Pain After Retroperitoneal Laparoscopic Donor Nephrectomy: A Study on Neuroinflammation and Neurophysiology

NOT YET RECRUITING
NCT07037992Phase NAIndonesia UniversityStarted 2025-07-01
injection of local anesthesia solutionInjection of normal saline
PreDiabetesDysbiosisEndotoxemia

Assessing Gut Microbiota Mediated Health Outcomes of Whole Wheat and Its Major Bioactive Components

ACTIVE NOT RECRUITING
NCT05318183Phase NAOhio State UniversityStarted 2022-01-27
Whole Wheat BreadWhite Bread (control)
AsthmaAtherosclerosisMetabolic Syndrome

Study of the Effect of Innate on the Inflammatory Response to Endotoxin

RECRUITING
NCT01143480National Institute of Environmental Health Sciences (NIEHS)Started 2012-07-30
Coronary Heart Disease

Adipose Tissue and Inflammation in Coronary Heart Disease

ACTIVE NOT RECRUITING
NCT02760914Oslo University HospitalStarted 2016-06
PeriodontitisEnd-stage Renal Disease

The Role of Toll-like Receptor-4 in Periodontitis Patients With End-stage Renal Disease in a Sample of Egyptian Population

RECRUITING
NCT06755372Ain Shams UniversityStarted 2025-04-01
Inflammatory Cytokine Expression

Effects of Propolis Flavonoids on TLR4 Signaling and Inflammation in Human PDLSCs In Vitro

NOT YET RECRUITING
NCT07367451Cairo UniversityStarted 2026-01-25
Flavonoid extract from propolisno medication added
Host-Pathogen Interactions

Genomic Approaches to Dissect Human Host-pathogen Interactions in the Amazonian Rainforest

NOT YET RECRUITING
NCT05981378Universidad Peruana Cayetano HerediaStarted 2024-08-02
Maternal ObesityObesity

The Developmental Origins of Obesity

RECRUITING
NCT06981676Phase NAPontificia Universidad Catolica de ChileStarted 2023-07-25
DHA and EPA
Alzheimer Disease (AD)

Tributyrin Treatment in Mild Alzheimer Disease: Assessment of Butyrate Effects Via the Gut-Brain

NOT YET RECRUITING
NCT06797817Phase PHASE3Universidad de AlmeriaStarted 2026-06-01
tributyrinPlacebo
Clinical Literature
Landmark / reviewRecent case evidence