MC5R

Chr 18

melanocortin 5 receptor

Also known as: MC2

The MC5R protein is a G protein-coupled receptor that binds melanocyte-stimulating hormones and ACTH, stimulating cAMP signaling pathways and playing essential roles in immune response, temperature regulation, and exocrine gland function. Mutations in MC5R have not been definitively associated with human disease, though the protein's functions suggest potential involvement in thermoregulatory, immune, or glandular disorders. The gene shows very low constraint against loss-of-function variants, indicating that complete loss of MC5R function may be well-tolerated in humans.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
9
Pubs (1 yr)
85
P/LP submissions
0%
P/LP missense
1.84
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryMC5R
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
81 unique Pathogenic / Likely Pathogenic· 60 VUS of 147 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.84LOEUF
pLI 0.000
Z-score -0.21
OE 1.10 (0.591.84)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.07Z-score
OE missense 0.99 (0.881.11)
202 obs / 204.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.10 (0.591.84)
00.351.4
Missense OE0.99 (0.881.11)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 6 / 5.5Missense obs/exp: 202 / 204.9Syn Z: -0.10
DN
0.76top 25%
GOF
0.82top 10%
LOF
0.2777th %ile

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

GOFprediction above median
DNprediction 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

147 submitted variants in ClinVar

Classification Summary

Pathogenic80
Likely Pathogenic1
VUS60
Likely Benign4
Benign1
80
Pathogenic
1
Likely Pathogenic
60
VUS
4
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
80
0
80
Likely Pathogenic
0
0
1
0
1
VUS
0
51
9
0
60
Likely Benign
0
4
0
0
4
Benign
0
0
0
1
1
Total055901146

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

View in ClinVar →

Protein Context — Lollipop Plot

MC5R · protein map & ClinVar variants

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

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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