PIGS

Chr 17AR

phosphatidylinositol glycan anchor biosynthesis class S

Also known as: DEE95, GPIBD18, PIG-S

This gene encodes a component of the GPI-anchor transamidase complex that catalyzes the attachment of GPI anchors to proteins in the endoplasmic reticulum, enabling these proteins to be anchored to cell surfaces. Biallelic mutations cause developmental and epileptic encephalopathy 95, inherited in an autosomal recessive pattern. The gene is highly constrained against loss-of-function variants (LOEUF 0.769), reflecting its essential role in cellular function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.771 OMIM phenotype
Clinical SummaryPIGS
🧬
Gene-Disease Validity (ClinGen)
glycosylphosphatidylinositol biosynthesis defect 18 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
5 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.77LOEUF
pLI 0.000
Z-score 2.45
OE 0.47 (0.300.77)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.78Z-score
OE missense 0.88 (0.800.97)
285 obs / 324.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.47 (0.300.77)
00.351.4
Missense OE0.88 (0.800.97)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 12 / 25.3Missense obs/exp: 285 / 324.5Syn Z: 0.17

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PIGS · 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

Hepatic Failure

Ex Vivo Perfusion of Gene-Edited Porcine Livers in Patients With Severe Hepatic Failure

ACTIVE NOT RECRUITING
NCT07519005Phase EARLY_PHASE1Xijing HospitalStarted 2026-01-31
Ex vivo perfusion of six-gene-edited porcine liverMulti-drug immunosuppressive therapy
Prostate Adenocarcinoma

Two Studies for Patients With Unfavorable Intermediate Risk Prostate Cancer Testing Less Intense Treatment for Patients With a Low Gene Risk Score and Testing a More Intense Treatment for Patients With a Higher Gene Risk Score, The Guidance Trial

ACTIVE NOT RECRUITING
NCT05050084Phase PHASE3NRG OncologyStarted 2021-12-06
BicalutamideBuserelinDarolutamide
Prostate AdenocarcinomaStage III Prostate Cancer AJCC v8Stage IVA Prostate Cancer AJCC v8

Two Studies for Patients With High Risk Prostate Cancer Testing Less Intense Treatment for Patients With a Low Gene Risk Score and Testing a More Intense Treatment for Patients With a High Gene Risk Score, The PREDICT-RT Trial

ACTIVE NOT RECRUITING
NCT04513717Phase PHASE3NRG OncologyStarted 2021-01-21
ApalutamideBicalutamideBiospecimen Collection
B-Cell Non-Hodgkin LymphomaHistiocytic and Dendritic Cell NeoplasmMyelodysplastic Syndrome

A Vaccine (VSV-hIFNβ-NIS) With or Without Cyclophosphamide and Combinations of Ipilimumab, Nivolumab, and Cemiplimab in Treating Relapsed or Refractory Multiple Myeloma, Acute Myeloid Leukemia or Lymphoma

RECRUITING
NCT03017820Phase PHASE1Mayo ClinicStarted 2017-04-04
Biopsy ProcedureBiospecimen CollectionBone Marrow Biopsy
Gastric Cancer Peritoneal Metastases

PIPAC in Gastric Peritoneal Metastasis

ENROLLING BY INVITATION
NCT07517419Phase PHASE2Chinese University of Hong KongStarted 2021-10-11
Cisplatin and Doxorubicin
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗