MARVELD2

Chr 5AR

MARVEL domain containing 2

Also known as: DFNB49, MARVD2, MRVLDC2, Tric

The protein forms tricellular tight junctions and epithelial barriers, particularly in the organ of Corti where it separates the endolymphatic and perilymphatic spaces and is required for hair cell survival. Mutations cause autosomal recessive deafness (DFNB49), typically presenting as congenital or early-onset hearing loss. The gene shows low constraint to loss-of-function variation (LOEUF 1.18), consistent with its autosomal recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismARLOEUF 1.181 OMIM phenotype
Clinical SummaryMARVELD2
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Gene-Disease Validity (ClinGen)
nonsyndromic genetic hearing loss · 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
1.18LOEUF
pLI 0.000
Z-score 0.85
OE 0.82 (0.581.18)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.20Z-score
OE missense 0.97 (0.881.06)
308 obs / 317.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.82 (0.581.18)
00.351.4
Missense OE0.97 (0.881.06)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 21 / 25.6Missense obs/exp: 308 / 317.9Syn Z: 0.31
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongMARVELD2-related deafnessLOFAR

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

DN
0.6937th %ile
GOF
0.6639th %ile
LOF
0.4332th %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

MARVELD2 · 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