LMO7

Chr 13

LIM domain 7

Also known as: FBX20, FBXO20, LMO7b, LOMP

The LMO7 protein contains calponin homology, PDZ, and LIM domains that mediate protein-protein interactions. Mutations cause autosomal dominant Emery-Dreifuss muscular dystrophy and dilated cardiomyopathy, affecting both skeletal and cardiac muscle. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic.

OMIMResearchSummary from RefSeq
MultiplemechanismLOEUF 0.41
Clinical SummaryLMO7
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.41LOEUF
pLI 0.000
Z-score 5.76
OE 0.29 (0.200.41)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
-0.17Z-score
OE missense 1.02 (0.961.08)
752 obs / 739.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.29 (0.200.41)
00.351.4
Missense OE1.02 (0.961.08)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 22 / 76.3Missense obs/exp: 752 / 739.1Syn Z: -0.32
DN
0.6842th %ile
GOF
0.6443th %ile
LOF
0.4234th %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

LMO7 · 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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