PI4K2A

Chr 10AR

phosphatidylinositol 4-kinase type 2 alpha

Also known as: NEDMSB, PI4KII, PIK42A

Phosphatidylinositolpolyphosphates (PtdInsPs) are centrally involved in many biologic processes, ranging from cell growth and organization of the actin cytoskeleton to endo- and exocytosis. PI4KII phosphorylates PtdIns at the D-4 position, an essential step in the biosynthesis of PtdInsPs (Barylko et al., 2001 [PubMed 11244087]).[supplied by OMIM, Mar 2008]

OMIMResearchGenerating clinical summary…
ARLOEUF 0.401 OMIM phenotype
Clinical SummaryPI4K2A
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.80) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
3 unique Pathogenic / Likely Pathogenic· 45 VUS of 53 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.40LOEUF
pLI 0.804
Z-score 3.67
OE 0.17 (0.090.40)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
2.89Z-score
OE missense 0.50 (0.430.57)
129 obs / 260.2 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.17 (0.090.40)
00.351.4
Missense OE?0.50 (0.430.57)
00.61.4
Synonymous OE?0.75
01.21.6
LoF obs/exp: 4 / 23.0Missense obs/exp: 129 / 260.2Syn Z: 1.97

ClinVar Variant Classifications

53 submitted variants in ClinVar

Classification Summary

Pathogenic3
VUS45
3
Pathogenic
45
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
0
0
0
3
Likely Pathogenic
0
0
0
0
0
VUS
0
45
0
0
45
Likely Benign
0
0
0
0
0
Benign
0
0
0
0
0
Total3450048

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

View in ClinVar →

18 pathogenic / likely-pathogenic (of 25) ClinVar copy-number / structural variants overlap PI4K2A — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

PI4K2A · 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.

Search ClinicalTrials.gov →