BCO1

Chr 16AD

beta-carotene oxygenase 1

Also known as: BCDO, BCDO1, BCMO, BCMO1, BCO

The encoded enzyme catalyzes the symmetric cleavage of beta-carotene into retinal, serving as a key step in vitamin A metabolism. Mutations cause hypercarotenemia and vitamin A deficiency with autosomal dominant inheritance. The gene shows minimal constraint against loss-of-function variants, suggesting tolerance to protein-disrupting mutations.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismADLOEUF 1.181 OMIM phenotype
Clinical SummaryBCO1
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
1.18LOEUF
pLI 0.000
Z-score 0.87
OE 0.81 (0.571.18)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.90Z-score
OE missense 1.15 (1.051.25)
346 obs / 302.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.81 (0.571.18)
00.351.4
Missense OE1.15 (1.051.25)
00.61.4
Synonymous OE1.21
01.21.6
LoF obs/exp: 20 / 24.6Missense obs/exp: 346 / 302.1Syn Z: -1.88
DN
0.5772th %ile
GOF
0.6052th %ile
LOF
0.3843th %ile

The Badonyi & Marsh model scores gain-of-function highest, but genomic evidence most strongly supports loss-of-function (haploinsufficiency) as the primary mechanism.

LOF1 literature citation

Literature Evidence

LOFThe mutation was not identified in 60 control chromosomes. The findings were consistent with a loss-of-function mutation resulting in haploinsufficiency.PMID:17951468

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BCO1 · 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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