GRM5

Chr 11

glutamate metabotropic receptor 5

Also known as: GPRC1E, MGLUR5, PPP1R86, mGlu5

The protein is a metabotropic glutamate receptor that activates phosphatidylinositol-calcium second messenger signaling and regulates synaptic plasticity and neural network activity. Mutations cause developmental and epileptic encephalopathy with an autosomal dominant inheritance pattern. This gene is highly constrained against loss-of-function variants (pLI = 1.0, LOEUF = 0.25), indicating that functional copies are critical for normal development.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.25
Clinical SummaryGRM5
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
18 unique Pathogenic / Likely Pathogenic· 129 VUS of 203 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.25LOEUF
pLI 0.999
Z-score 5.24
OE 0.12 (0.060.25)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.21Z-score
OE missense 0.65 (0.600.70)
425 obs / 656.2 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.12 (0.060.25)
00.351.4
Missense OE0.65 (0.600.70)
00.61.4
Synonymous OE1.18
01.21.6
LoF obs/exp: 5 / 41.4Missense obs/exp: 425 / 656.2Syn Z: -2.25
DN
0.5081th %ile
GOF
0.6735th %ile
LOF
0.63top 25%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function 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 loss-of-function (haploinsufficiency). Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFLOEUF 0.25
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. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

203 submitted variants in ClinVar

Classification Summary

Pathogenic17
Likely Pathogenic1
VUS129
Likely Benign28
Benign15
Conflicting1
17
Pathogenic
1
Likely Pathogenic
129
VUS
28
Likely Benign
15
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
17
0
17
Likely Pathogenic
0
0
1
0
1
VUS
0
124
5
0
129
Likely Benign
0
1
3
24
28
Benign
0
2
1
12
15
Conflicting
1
Total01272736191

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

View in ClinVar →

Protein Context — Lollipop Plot

GRM5 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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