KDM8

Chr 16

lysine demethylase 8

Also known as: JMJD5

KDM8 encodes a bifunctional enzyme that acts as both an endopeptidase cleaving histone N-terminal tails and a 2-oxoglutarate-dependent monooxygenase, playing critical roles in transcriptional regulation, mitosis, cell cycle progression, and circadian gene expression. The gene shows very low constraint to loss-of-function variants (pLI = 0.00003, LOEUF = 0.836), suggesting tolerance to haploinsufficiency. No established Mendelian diseases have been associated with KDM8 mutations in pediatric populations.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.84
Clinical SummaryKDM8
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
0.84LOEUF
pLI 0.000
Z-score 2.14
OE 0.50 (0.320.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.61Z-score
OE missense 0.90 (0.810.99)
247 obs / 275.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.50 (0.320.84)
00.351.4
Missense OE0.90 (0.810.99)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 11 / 21.8Missense obs/exp: 247 / 275.7Syn Z: -0.04
DN
0.6551th %ile
GOF
0.5465th %ile
LOF
0.2678th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KDM8 · 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.

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Clinical Literature
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