GPR158

Chr 10

G protein-coupled receptor 158

Also known as: mGlyR

GPR158 encodes a metabotropic glycine receptor that controls synapse formation and function in the brain by regulating G protein signaling and cAMP levels, particularly affecting neuronal excitability in the prefrontal cortex and hippocampus. Mutations cause autosomal recessive neurodevelopmental disorders characterized by intellectual disability, developmental delay, and behavioral abnormalities with childhood onset. This gene is highly constrained against loss-of-function variants (LOEUF 0.467), indicating that biallelic mutations likely cause severe phenotypes.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
14
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.47
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryGPR158
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.47LOEUF
pLI 0.001
Z-score 4.63
OE 0.31 (0.210.47)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.38Z-score
OE missense 0.96 (0.901.02)
645 obs / 672.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.31 (0.210.47)
00.351.4
Missense OE0.96 (0.901.02)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 16 / 52.0Missense obs/exp: 645 / 672.5Syn Z: 0.12
DN
0.6839th %ile
GOF
0.6541th %ile
LOF
0.4038th %ile

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

DNprediction above median
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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GPR158 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗