MAB21L2

Chr 4ADAR

mab-21 like 2

Also known as: MCOPS14, MCSKS14

MAB21L2 encodes a nuclear protein required for normal embryonic eye development and is similar to C. elegans cell fate-determining genes downstream of TGF-beta signaling. Mutations cause microphthalmia/coloboma and skeletal dysplasia syndrome with both autosomal dominant and autosomal recessive inheritance patterns reported. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.549), reflecting its essential role in early developmental processes affecting ocular and skeletal systems.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismAD/ARLOEUF 0.551 OMIM phenotype
Clinical SummaryMAB21L2
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Gene-Disease Validity (ClinGen)
colobomatous microphthalmia-rhizomelic dysplasia syndrome · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.21) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.55LOEUF
pLI 0.438
Z-score 2.74
OE 0.21 (0.100.55)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.96Z-score
OE missense 0.46 (0.390.54)
110 obs / 238.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.21 (0.100.55)
00.351.4
Missense OE0.46 (0.390.54)
00.61.4
Synonymous OE0.82
01.21.6
LoF obs/exp: 3 / 14.1Missense obs/exp: 110 / 238.5Syn Z: 1.53

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MAB21L2 · 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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