KDM4A

Chr 1

lysine demethylase 4A

Also known as: JHDM3A, JMJD2, JMJD2A, TDRD14A

This histone demethylase specifically removes trimethyl groups from lysine-9 and lysine-36 residues of histone H3, regulating gene expression through chromatin modification. Mutations cause autosomal dominant neurodevelopmental disorder with intellectual disability, seizures, and behavioral abnormalities. The gene is extremely intolerant to loss-of-function variants, indicating that proper dosage is critical for normal neurodevelopment.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.16
Clinical SummaryKDM4A
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
8 unique Pathogenic / Likely Pathogenic· 109 VUS of 159 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.16LOEUF
pLI 1.000
Z-score 6.62
OE 0.07 (0.030.16)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.68Z-score
OE missense 0.58 (0.530.63)
351 obs / 606.0 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.07 (0.030.16)
00.351.4
Missense OE0.58 (0.530.63)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 4 / 58.7Missense obs/exp: 351 / 606.0Syn Z: 0.80
DN
0.2399th %ile
GOF
0.3193th %ile
LOF
0.74top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.16

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

159 submitted variants in ClinVar

Classification Summary

Pathogenic7
Likely Pathogenic1
VUS109
Likely Benign6
Benign6
7
Pathogenic
1
Likely Pathogenic
109
VUS
6
Likely Benign
6
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
7
0
7
Likely Pathogenic
0
0
1
0
1
VUS
0
105
4
0
109
Likely Benign
0
3
0
3
6
Benign
0
1
1
4
6
Total0109137129

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

View in ClinVar →

Protein Context — Lollipop Plot

KDM4A · 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
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