MC4R

Chr 18ADAR

melanocortin 4 receptor

Also known as: BMIQ20

The melanocortin 4 receptor is a G protein-coupled receptor in the hypothalamus that binds melanocyte-stimulating hormones and ACTH to regulate energy homeostasis by suppressing appetite and food intake. Mutations cause obesity that can follow either autosomal dominant or autosomal recessive inheritance patterns. The gene shows low constraint against loss-of-function variants (pLI 0.0009, LOEUF 1.6), consistent with its association with obesity rather than severe developmental disorders.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

{Obesity, resistance to (BMIQ20)}MIM #618406
ADAR
Obesity (BMIQ20)MIM #618406
ADAR
10
Active trials
180
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.60
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryMC4R
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
10 active or recruiting trials — potential therapeutic options may be available
📖
GeneReview available — MC4R
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.60LOEUF
pLI 0.001
Z-score 0.48
OE 0.79 (0.411.60)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.01Z-score
OE missense 1.21 (1.081.36)
218 obs / 179.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.79 (0.411.60)
00.351.4
Missense OE1.21 (1.081.36)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 5 / 6.3Missense obs/exp: 218 / 179.9Syn Z: -0.85
DN
0.79top 25%
GOF
0.84top 5%
LOF
0.2485th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function, dominant-negative and loss-of-function). 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 · 1 literature citation
DNprediction above median
LOF1 literature citation

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

GOFHuman Gain-of-Function MC4R Variants Show Signaling Bias and Protect against Obesity.PMID:31002796
LOFThis case supports haploinsufficiency of MC4-R as it describes a MC4-R deficiency phenotype in a patient heterozygous for a full MC4R gene deletion.PMID:27738543

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MC4R · 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

Obesity and Obesity-related Medical Conditions

Precision Nutrition Technologies for Obesity Management

NOT YET RECRUITING
NCT07101133Phase NAYuan HeStarted 2025-07-27
Control group: Energy-limited balanced diet group
Obesity Prevention

Assessing Obesity Related Risk Factors in Adult Population: A Genetic and Behavioral Study

ENROLLING BY INVITATION
NCT07341841Phase NAAugusta UniversityStarted 2026-01-01
Diet and exerciseLifestyle InterventionExercise
Obesity

Study to Investigate Genetic Causes of Severe Early Childhood Onset Obesity.

RECRUITING
NCT01998750Columbia UniversityStarted 2014-02
Obesity

Intensive Weight Loss Intervention Versus Usual Care for Adults With Severe and Complex Obesity

RECRUITING
NCT06321458Phase NACarsten DirksenStarted 2024-04-29
Intensive weight loss interventionUsual care
Obesity and Obesity-related Medical ConditionsCardiovascular RiskGenetics

Cardiometabolic Risk of Obese Subjects: Cross-sectional Study

RECRUITING
NCT06714058IRCCS Azienda Ospedaliero-Universitaria di BolognaStarted 2024-11-30
Obesity

Intensive Weight Loss Intervention Versus Usual Care for Adults With Obesity

ACTIVE NOT RECRUITING
NCT06321432Phase NACarsten DirksenStarted 2024-06-05
Intensive weight loss interventionUsual care
Monogenic Obesity

Efficacy of Semaglutide s.c. Once-weekly on Weight Loss and Management in Adolescents With Monogenic Obesity in Clinical Practice

RECRUITING
NCT07302802Prof. Dr. Martin WabitschStarted 2025-12-01
Semaglutide (administered by PDS290 pen-injector)
Morbid ObesityBariatric SurgeryTelerehabilitation

Exercise to Fight Obesity

RECRUITING
NCT06934681Phase NAInstituto de Investigacion Sanitaria La FeStarted 2025-05-19
Usual Care GroupControlled exercise with telerehabilitation
ObesityGenetic Obesity

EMANATE: A Study of Setmelanotide in Patients With Specific Gene Variants in the MC4R Pathway

ACTIVE NOT RECRUITING
NCT05093634Phase PHASE3Rhythm Pharmaceuticals, Inc.Started 2021-12-10
SetmelanotidePlacebo
Obesity

Intensive Weight Loss Intervention Versus Bariatric Surgery for Adults With Severe and Complex Obesity: the LightBAR Randomised Trial

RECRUITING
NCT06309238Phase NACarsten DirksenStarted 2024-05-08
Intensive weight loss interventionBariatric surgery
Clinical Literature
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