MBOAT7

Chr 19AR

membrane bound acylglycerophosphatidylinositol O-acyltransferase MBOAT7

Also known as: BB1, LENG4, LPIAT, LPIAT1, LPLAT, LPLAT11, LRC4, MBOA7

This lysophosphatidylinositol acyltransferase catalyzes the transfer of acyl groups (preferentially arachidonoyl-CoA) to lysophosphatidylinositol during phospholipid remodeling in the Lands cycle, regulating cellular arachidonic acid levels and triglyceride metabolism. Biallelic mutations cause autosomal recessive intellectual developmental disorder. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.581).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.581 OMIM phenotype
Clinical SummaryMBOAT7
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.28) despite low pLI — interpret in context.
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Clinical Trials
4 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.58LOEUF
pLI 0.113
Z-score 2.85
OE 0.28 (0.140.58)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.44Z-score
OE missense 0.93 (0.841.02)
274 obs / 295.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.28 (0.140.58)
00.351.4
Missense OE0.93 (0.841.02)
00.61.4
Synonymous OE1.17
01.21.6
LoF obs/exp: 5 / 18.1Missense obs/exp: 274 / 295.3Syn Z: -1.51
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongMBOAT7-related intellectual disability accompanied by epilepsy and autistic featuresLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6743th %ile
GOF
0.76top 25%
LOF
0.2776th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MBOAT7 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗