MRAP2

Chr 6AD

melanocortin 2 receptor accessory protein 2

The protein modulates melanocortin receptor 4 (MC4R) signaling, which controls energy homeostasis and body weight regulation by increasing the receptor's ligand sensitivity and cAMP generation. Mutations cause severe early-onset obesity through disrupted energy balance. The gene shows tolerance to loss-of-function variants (low constraint), and inheritance pattern has not been definitively established in the limited reported cases.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismADLOEUF 1.881 OMIM phenotype
Clinical SummaryMRAP2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
4 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.88LOEUF
pLI 0.000
Z-score -0.67
OE 1.27 (0.751.88)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.31Z-score
OE missense 0.92 (0.781.08)
99 obs / 108.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.27 (0.751.88)
00.351.4
Missense OE0.92 (0.781.08)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 9 / 7.1Missense obs/exp: 99 / 108.0Syn Z: -0.25
DN
0.7034th %ile
GOF
0.80top 10%
LOF
0.3163th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). The Badonyi & Marsh model scores gain-of-function highest among its predictions, but genomic evidence (constraint, ClinVar variant spectrum, and literature) most strongly supports dominant-negative. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median · 1 literature citation
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.

Literature Evidence

DNRegulation of G protein-coupled receptor signaling: specific dominant-negative effects of melanocortin 2 receptor accessory protein 2.PMID:20371771

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MRAP2 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗