GLYR1

Chr 16

glyoxylate reductase 1 homolog

Also known as: BM045, HIBDL, N-PAC, NP60, NPAC, hNDF

GLYR1 encodes a chromatin remodeling factor that destabilizes nucleosomes during transcriptional activation and regulates gene expression through histone modifications and interactions with histone-modifying enzymes. Mutations cause autosomal dominant neurodevelopmental disorder with developmental delay, intellectual disability, and variable features including seizures and behavioral abnormalities. The gene is highly constrained against loss-of-function variants (pLI = 1.0, LOEUF = 0.195), reflecting its essential role in transcriptional regulation.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.20
Clinical SummaryGLYR1
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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.20LOEUF
pLI 1.000
Z-score 4.94
OE 0.06 (0.020.20)
Highly constrained

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

Missense Constraint
2.34Z-score
OE missense 0.64 (0.570.72)
215 obs / 335.9 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.06 (0.020.20)
00.351.4
Missense OE0.64 (0.570.72)
00.61.4
Synonymous OE1.42
01.21.6
LoF obs/exp: 2 / 32.3Missense obs/exp: 215 / 335.9Syn Z: -3.87
DN
0.3495th %ile
GOF
0.3193th %ile
LOF
0.78top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.20

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GLYR1 · 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
Open Research Assistant →