MAB21L1

Chr 13AR

mab-21 like 1

Also known as: CAGR1, COFG, Nbla00126

MAB21L1 encodes a putative nucleotidyltransferase that binds single-stranded RNA and is required for normal embryonic development of the eye and other structures. Biallelic mutations cause cerebellar, ocular, craniofacial, and genital syndrome, which follows autosomal recessive inheritance. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.628), consistent with its role in multiple developmental processes.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.631 OMIM phenotype
Clinical SummaryMAB21L1
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Gene-Disease Validity (ClinGen)
cerebellar, ocular, craniofacial, and genital syndrome · ARStrong

Strong evidence — appropriate for clinical testing

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.24) despite low pLI — interpret in context.
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ClinVar Variants
62 unique Pathogenic / Likely Pathogenic· 37 VUS of 106 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.63LOEUF
pLI 0.286
Z-score 2.47
OE 0.24 (0.110.63)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.10Z-score
OE missense 0.78 (0.680.89)
150 obs / 193.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.24 (0.110.63)
00.351.4
Missense OE0.78 (0.680.89)
00.61.4
Synonymous OE1.28
01.21.6
LoF obs/exp: 3 / 12.4Missense obs/exp: 150 / 193.2Syn Z: -2.02

ClinVar Variant Classifications

106 submitted variants in ClinVar

Classification Summary

Pathogenic57
Likely Pathogenic5
VUS37
Likely Benign6
57
Pathogenic
5
Likely Pathogenic
37
VUS
6
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
4
2
51
0
57
Likely Pathogenic
1
1
3
0
5
VUS
0
34
3
0
37
Likely Benign
0
1
0
5
6
Benign
0
0
0
0
0
Total538575105

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

MAB21L1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC