LYSMD4

Chr 15

LysM domain containing 4

LYSMD4 encodes a protein predicted to be located in cellular membranes, though its specific function remains poorly characterized. Mutations cause autosomal recessive developmental delay, intellectual disability, and seizures with onset in infancy or early childhood. The gene shows high constraint against loss-of-function variants (pLI = 0.0006), suggesting that complete protein loss is not well tolerated.

ResearchSummary from RefSeq
MultiplemechanismLOEUF 1.33
Clinical SummaryLYSMD4
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
1.33LOEUF
pLI 0.001
Z-score 0.89
OE 0.68 (0.371.33)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.00Z-score
OE missense 1.00 (0.881.14)
170 obs / 170.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.68 (0.371.33)
00.351.4
Missense OE1.00 (0.881.14)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 6 / 8.9Missense obs/exp: 170 / 170.1Syn Z: 0.05
DN
0.7131th %ile
GOF
0.6735th %ile
LOF
0.3358th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LYSMD4 · 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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