MINAR2

Chr 5AR

membrane integral NOTCH2 associated receptor 2

Also known as: DFNB120, KIAA1024L

This protein binds cholesterol and regulates cholesterol distribution and homeostasis in hair cells, and may function in angiogenesis. Mutations cause autosomal recessive nonsyndromic deafness (DFNB120). The gene is not highly constrained against loss-of-function variants, consistent with its autosomal recessive inheritance pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 1.481 OMIM phenotype
Clinical SummaryMINAR2
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
1.48LOEUF
pLI 0.001
Z-score 0.69
OE 0.72 (0.371.48)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.67Z-score
OE missense 0.79 (0.650.97)
67 obs / 84.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.72 (0.371.48)
00.351.4
Missense OE0.79 (0.650.97)
00.61.4
Synonymous OE0.74
01.21.6
LoF obs/exp: 5 / 7.0Missense obs/exp: 67 / 84.4Syn Z: 1.14
DN
0.83top 10%
GOF
0.82top 10%
LOF
0.3068th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MINAR2 · 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