MTNR1A

Chr 4

melatonin receptor 1A

Also known as: MEL-1A-R, MT1

This protein is a high-affinity G-protein coupled receptor for melatonin that mediates circadian rhythm and reproductive functions, primarily localized to the hypothalamic suprachiasmatic nucleus and pituitary. Mutations cause autosomal recessive nonsyndromic intellectual disability and may be associated with sleep disorders given the gene's role in melatonin signaling. The gene shows very low constraint against loss-of-function variants (pLI near zero), consistent with recessive inheritance requiring biallelic mutations for disease manifestation.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
21
Pubs (1 yr)
116
P/LP submissions
0%
P/LP missense
1.77
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryMTNR1A
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
115 unique Pathogenic / Likely Pathogenic· 71 VUS of 202 total submissions
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GeneReview available — MTNR1A
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
1.77LOEUF
pLI 0.000
Z-score -0.22
OE 1.08 (0.651.77)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.09Z-score
OE missense 0.98 (0.871.10)
198 obs / 201.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.08 (0.651.77)
00.351.4
Missense OE0.98 (0.871.10)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 9 / 8.3Missense obs/exp: 198 / 201.7Syn Z: -0.01
DN
0.81top 10%
GOF
0.85top 5%
LOF
0.2288th %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

202 submitted variants in ClinVar

Classification Summary

Pathogenic103
Likely Pathogenic12
VUS71
Likely Benign6
Benign7
Conflicting1
103
Pathogenic
12
Likely Pathogenic
71
VUS
6
Likely Benign
7
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
103
0
103
Likely Pathogenic
0
0
12
0
12
VUS
0
38
33
0
71
Likely Benign
0
2
2
2
6
Benign
0
2
3
2
7
Conflicting
1
Total0421534200

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

View in ClinVar →

Protein Context — Lollipop Plot

MTNR1A · 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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