PGAP4

Chr 9

post-GPI attachment to proteins GalNAc transferase 4

Also known as: C9orf125, TMEM246

PGAP4 encodes a Golgi-resident glycosyltransferase that catalyzes N-acetylgalactosamine transfer to the first mannose of glycosylphosphatidylinositol (GPI) anchors during GPI-anchor maturation. Mutations cause autosomal recessive intellectual disability with seizures, abnormal gait, and delayed speech development. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.696), and patients typically present in early childhood with developmental delays and neurological symptoms.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.70
Clinical SummaryPGAP4
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.30) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.70LOEUF
pLI 0.094
Z-score 2.34
OE 0.30 (0.150.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.42Z-score
OE missense 0.74 (0.660.84)
177 obs / 238.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.30 (0.150.70)
00.351.4
Missense OE0.74 (0.660.84)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 4 / 13.1Missense obs/exp: 177 / 238.6Syn Z: -0.31

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PGAP4 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found