PAGR1

Chr 16

PAXIP1 associated glutamate rich protein 1

Also known as: C16orf53, GAS, PA1

PAGR1 encodes a transcriptional cofactor that associates with histone methyltransferase complexes and regulates estrogen receptor signaling and cell cycle progression. Mutations cause autosomal dominant neurodevelopmental disorder with developmental delay, intellectual disability, and behavioral abnormalities. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.538), consistent with its role in essential cellular processes.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.54
Clinical SummaryPAGR1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.74) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.54LOEUF
pLI 0.743
Z-score 2.44
OE 0.11 (0.040.54)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.63Z-score
OE missense 0.86 (0.740.99)
129 obs / 150.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.11 (0.040.54)
00.351.4
Missense OE0.86 (0.740.99)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 1 / 8.8Missense obs/exp: 129 / 150.9Syn Z: -0.32

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PAGR1 · 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.

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Clinical Literature
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