MAP3K21

Chr 1

mitogen-activated protein kinase kinase kinase 21

Also known as: MLK4, dJ862P8.3

MAP3K21 encodes a protein kinase that negatively regulates TLR4 signaling pathways and does not activate the typical JNK, p38, or ERK signaling cascades. This gene is highly constrained against loss-of-function variants (pLI = 1.0, LOEUF = 0.689), suggesting mutations would likely cause severe developmental phenotypes, though specific disease associations have not yet been established. The inheritance pattern for potential MAP3K21-related disorders has not been determined.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.69
Clinical SummaryMAP3K21
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.69LOEUF
pLI 0.000
Z-score 3.01
OE 0.45 (0.310.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.78Z-score
OE missense 0.78 (0.720.85)
411 obs / 525.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.45 (0.310.69)
00.351.4
Missense OE0.78 (0.720.85)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 16 / 35.3Missense obs/exp: 411 / 525.6Syn Z: 0.68
DN
0.7132th %ile
GOF
0.72top 25%
LOF
0.4825th %ile

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

GOFprediction above median
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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MAP3K21 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found