OCA2

Chr 15AR

OCA2 melanosomal transmembrane protein

Also known as: BEY, BEY1, BEY2, BOCA, D15S12, EYCL, EYCL2, EYCL3

This gene encodes the human homolog of the mouse p (pink-eyed dilution) gene. The encoded protein is believed to be an integral membrane protein involved in small molecule transport, specifically tyrosine, which is a precursor to melanin synthesis. It is involved in mammalian pigmentation, where it may control skin color variation and act as a determinant of brown or blue eye color. Mutations in this gene result in type 2 oculocutaneous albinism. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2014]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.867 OMIM phenotypes
Clinical SummaryOCA2
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Gene-Disease Validity (ClinGen)
oculocutaneous albinism type 2 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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?
0.86LOEUF
pLI 0.000
Z-score 2.32
OE 0.62 (0.460.86)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.83Z-score
OE missense 1.11 (1.031.19)
535 obs / 483.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.62 (0.460.86)
00.351.4
Missense OE?1.11 (1.031.19)
00.61.4
Synonymous OE?1.13
01.21.6
LoF obs/exp: 27 / 43.5Missense obs/exp: 535 / 483.5Syn Z: -1.42
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveOCA2-related oculocutaneous albinismLOFAR

This gene — mechanism propensity

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

DN
0.78top 25%
GOF
0.73top 25%
LOF
0.2678th %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

OCA2 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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