OSBPL9

Chr 1

oxysterol binding protein like 9

Also known as: ORP-9, ORP9

The protein functions as a cholesterol transfer protein that regulates Golgi structure and exchanges phosphatidylserine for phosphatidylinositol-4-phosphate between the endoplasmic reticulum and trans-Golgi, which is critical for sphingomyelin synthesis. This gene is highly constrained against loss-of-function variants (pLI = 0.98, LOEUF = 0.31), but no established human disease has been definitively associated with OSBPL9 mutations to date.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.31
Clinical SummaryOSBPL9
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.31LOEUF
pLI 0.976
Z-score 5.25
OE 0.17 (0.100.31)
Highly constrained

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

Missense Constraint
2.66Z-score
OE missense 0.62 (0.560.69)
246 obs / 394.4 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.17 (0.100.31)
00.351.4
Missense OE0.62 (0.560.69)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 8 / 46.7Missense obs/exp: 246 / 394.4Syn Z: 0.69
DN
0.3792th %ile
GOF
0.4874th %ile
LOF
0.67top 25%

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

LOFprediction above median · LOEUF 0.31

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

OSBPL9 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC