PIK3R6

Chr 17

phosphoinositide-3-kinase regulatory subunit 6

Also known as: C17orf38, HsT41028, p84 PIKAP, p87(PIKAP), p87PIKAP

The PIK3R6 protein is a regulatory subunit of PI3K gamma, a lipid kinase that produces phosphatidylinositol 3,4,5-trisphosphate and mediates signaling downstream of G protein-coupled receptors. Mutations in PIK3R6 cause autosomal recessive immunodeficiency with recurrent infections, growth retardation, and neurodevelopmental delays. The gene shows very low constraint against loss-of-function variants, consistent with the recessive inheritance pattern observed in affected patients.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.46
Clinical SummaryPIK3R6
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
10 unique Pathogenic / Likely Pathogenic· 109 VUS of 143 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.46LOEUF
pLI 0.000
Z-score -0.68
OE 1.12 (0.871.46)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.13Z-score
OE missense 0.98 (0.911.06)
437 obs / 444.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.12 (0.871.46)
00.351.4
Missense OE0.98 (0.911.06)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 40 / 35.6Missense obs/exp: 437 / 444.4Syn Z: -0.24

ClinVar Variant Classifications

143 submitted variants in ClinVar

Classification Summary

Pathogenic10
VUS109
Likely Benign2
10
Pathogenic
109
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
10
0
10
Likely Pathogenic
0
0
0
0
0
VUS
0
106
3
0
109
Likely Benign
0
2
0
0
2
Benign
0
0
0
0
0
Total0108130121

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PIK3R6 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC