PIGB

Chr 15AR

phosphatidylinositol glycan anchor biosynthesis class B

Also known as: DEE80, EIEE80, GPI-MT-III, PIG-B

This gene encodes a transmembrane protein that is located in the endoplasmic reticulum and is involved in GPI-anchor biosynthesis. The glycosylphosphatidylinositol (GPI) anchor is a glycolipid found on many blood cells and serves to anchor proteins to the cell surface. This gene is thought to encode a member of a family of dolichol-phosphate-mannose (Dol-P-Man) dependent mannosyltransferases. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 1.061 OMIM phenotype
Clinical SummaryPIGB
🧬
Gene-Disease Validity (ClinGen)
developmental and epileptic encephalopathy, 80 · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.06LOEUF
pLI 0.000
Z-score 1.30
OE 0.73 (0.511.06)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.60Z-score
OE missense 0.90 (0.811.00)
239 obs / 266.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.73 (0.511.06)
00.351.4
Missense OE?0.90 (0.811.00)
00.61.4
Synonymous OE?0.96
01.21.6
LoF obs/exp: 20 / 27.3Missense obs/exp: 239 / 266.3Syn Z: 0.29
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitivePIGB-related inherited GPI biosynthesis defect with an axonal neuropathy and metabolic abnormalityLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7131th %ile
GOF
0.5071th %ile
LOF
0.3260th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PIGB · 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.

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