Genetic Information

Gene & Transcript Details

Gene
PIK3CA
Transcript
NM_006218.4 MANE Select
Total Exons
Reference Sequence
NC_000003.11
Alternative Transcripts
IDStatusDetails
NM_006218.2 Alternative 3724 nt | 158–3364
NM_006218.3 Alternative 9104 nt | 158–3364
NM_006218.4 MANE Select 9259 nt | 324–3530

Variant Details

HGVS Notation
NM_006218.4:c.2727C>G
Protein Change
F909L
Location
Exon 19 (Exon 19 of )
19
5'Exon Structure3'
Functional Consequence
Loss of Function
Alternate Identifiers

Clinical & Population Data

Population Frequency

gnomAD
Global Frequency
0.0 in 100,000
Extremely Rare
ACMG Criteria Applied PM2
This variant is absent or extremely rare in population databases (PM2 criteria applies).

ClinVar

Open
Classification
Unknown
0 publications
Clinical Statement

""

COSMIC Somatic Evidence

Open
COSMIC ID
COSM5624544
Recurrence
4 occurrences
PM1 Criteria
Not Applied
COSMIC Database Preview
COSMIC Preview
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Functional Impact & Domains

Functional Domain

Hotspot Status
Not a hotspot
Domain Summary
This variant is not located in a mutational hotspot or critical domain.
Related Variants in This Domain
No evidence of other pathogenic variants at this position in gene PIK3CA.

Functional Studies & Therapeutic Relevance

Functional Summary

The PIK3CA F909L variant has not been functionally characterized, and its effect on protein function is currently unknown.

Database Previews
OncoKB
OncoKB Preview
JAX-CKB
JAX-CKB Preview

Click on previews to view full database entries. External databases may require institutional access.

Computational Analysis

Pathogenicity Predictions

SpliceAI
Predictor Consensus
Mixed/VUS
PP3 Applied
No
REVEL Score
0.632
Threshold: ≥0.75 = PP3 applied

SpliceAI Scores

Window: ±500bp
Effect Type Score Position
- Acceptor Loss (AL) 0.03 -60 bp
- Donor Loss (DL) 0.01 63 bp
+ Acceptor Gain (AG) 0.0 -7 bp
+ Donor Gain (DG) 0.0 66 bp
High impact (≥0.5) Medium impact (0.2-0.49) Low impact (<0.2)

VCEP Guidelines

Applied ACMG/AMP Criteria (VCEP Specific)

Filter Criteria:
PVS1

PVS1 (Not Applied)

According to standard ACMG guidelines, the rule for PVS1 is: "Null variant (nonsense, frame-shift, canonical ±1/2 splice sites, initiation codon, single or multi-exon deletion) in a gene where loss of function is a known mechanism of disease." The evidence for this variant shows: it is a missense change (F909L), not a null variant. Therefore, this criterion is not applied.

PS1

PS1 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PS1 is: "Strong: same amino acid change as a previously established pathogenic variant regardless of nucleotide change." The evidence for this variant shows: no previously established pathogenic variant at amino acid F909. Therefore, this criterion is not applied.

PS2

PS2 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PS2 is: "Moderate or Strong evidence of de novo occurrence in a patient, with criteria for high-confidence somatic mutations." The evidence for this variant shows: no data on de novo occurrence. Therefore, this criterion is not applied.

PS3

PS3 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PS3 is: "Functional assay meets acceptability criteria set by BMVCEP (PMID:31892348) with quality metrics and controls." The evidence for this variant shows: no functional characterization data. Therefore, this criterion is not applied.

PS4

PS4 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PS4 is: "Aggregate case data absent from controls with phenotype point scoring (≥0.5 points for Supporting up to ≥16 for Very Strong)." The evidence for this variant shows: no reported clinical cases or phenotype data. Therefore, this criterion is not applied.

PM1

PM1 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PM1 is: "Supporting: residue affects critical functional domain as defined in Table 4." The evidence for this variant shows: F909 is not located in a known critical functional hotspot domain of PIK3CA. Therefore, this criterion is not applied.

PM2

PM2 (Supporting)

According to PIK3CA VCEP guidelines, the rule for PM2 is: "Supporting Absent/rare from controls in an ethnically-matched cohort population sample (≥1)." The evidence for this variant shows: allele not found in gnomAD or other population databases (MAF=0%). Therefore, this criterion is applied at Supporting strength because the variant is absent from population controls.

PM3

PM3 (Not Applied)

According to standard ACMG guidelines, the rule for PM3 is: "Detected in trans with a pathogenic variant for a recessive disorder." The evidence for this variant shows: PIK3CA‐related disorders are not inherited in a recessive manner and no trans data exist. Therefore, this criterion is not applied.

PM4

PM4 (Not Applied)

According to standard ACMG guidelines, the rule for PM4 is: "Protein length changes due to in-frame deletions/insertions in non-repetitive regions." The evidence for this variant shows: it is a missense substitution without length change. Therefore, this criterion is not applied.

PM5

PM5 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PM5 is: "Moderate: novel missense change at an amino acid residue where a different missense change is pathogenic." The evidence for this variant shows: no pathogenic variants reported at residue F909. Therefore, this criterion is not applied.

PM6

PM6 (Not Applied)

According to standard ACMG guidelines, the rule for PM6 is: "Assumed de novo without confirmation of paternity/maternity." The evidence for this variant shows: no de novo data. Therefore, this criterion is not applied.

PP1

PP1 (Not Applied)

According to standard ACMG guidelines, the rule for PP1 is: "Co‐segregation with disease in multiple affected family members." The evidence for this variant shows: no segregation data. Therefore, this criterion is not applied.

PP2

PP2 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for PP2 is: "Supporting: missense constraint computed in ExAC/gnomAD z-score >3.09." The evidence for this variant shows: gene‐level constraint z-score data not provided. Therefore, this criterion is not applied.

PP3

PP3 (Not Applied)

According to standard ACMG guidelines, the rule for PP3 is: "Multiple lines of computational evidence support a deleterious effect." The evidence for this variant shows: in silico predictions are mixed (REVEL 0.63, CADD benign, SpliceAI no splice impact). Therefore, this criterion is not applied.

PP4

PP4 (Not Applied)

According to standard ACMG guidelines, the rule for PP4 is: "Patient’s phenotype or family history is highly specific for a disease with a single genetic etiology." The evidence for this variant shows: no phenotype data provided. Therefore, this criterion is not applied.

PP5

PP5 (Not Applied)

According to standard ACMG guidelines, the rule for PP5 is: "Reputable source reports variant as pathogenic without available evidence." The evidence for this variant shows: not reported in ClinVar or other expert sources. Therefore, this criterion is not applied.

BA1

BA1 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BA1 is: "Stand Alone: allele frequency >0.0926%." The evidence for this variant shows: MAF=0% in population databases. Therefore, this criterion is not applied.

BS1

BS1 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BS1 is: "Strong: allele frequency >0.0185%." The evidence for this variant shows: MAF=0%. Therefore, this criterion is not applied.

BS2

BS2 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BS2 is: "Strong: ≥3 homozygotes or heterozygotes in well‐phenotyped individuals." The evidence for this variant shows: no homozygotes or well‐phenotyped observations. Therefore, this criterion is not applied.

BS3

BS3 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BS3 is: "Strong or Supporting functional studies show no damaging effect." The evidence for this variant shows: no functional assay data. Therefore, this criterion is not applied.

BS4

BS4 (Not Applied)

According to standard ACMG guidelines, the rule for BS4 is: "Lack of segregation in affected family members." The evidence for this variant shows: no segregation data. Therefore, this criterion is not applied.

BP1

BP1 (Not Applied)

According to standard ACMG guidelines, the rule for BP1 is: "Missense variant in gene where only truncating variants cause disease." The evidence for this variant shows: PIK3CA disease mechanism is gain-of-function, not solely truncating. Therefore, this criterion is not applied.

BP2

BP2 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BP2 is: "Supporting: variant observed in cis/trans with a known pathogenic variant." The evidence for this variant shows: no data on cis/trans observations. Therefore, this criterion is not applied.

BP3

BP3 (Not Applied)

According to standard ACMG guidelines, the rule for BP3 is: "In-frame deletions/insertions in repetitive regions without known function." The evidence for this variant shows: it is a missense substitution. Therefore, this criterion is not applied.

BP4

BP4 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BP4 is: "Supporting for synonymous/intronic/UTR variants when ≥2 splicing tools predict no impact." The evidence for this variant shows: it is a missense change, not eligible for BP4 under VCEP. Therefore, this criterion is not applied.

BP5

BP5 (Not Applied)

According to standard ACMG guidelines, the rule for BP5 is: "Variant found in a case with an alternate molecular basis for disease." The evidence for this variant shows: no alternate cause reported. Therefore, this criterion is not applied.

BP6

BP6 (Not Applied)

According to standard ACMG guidelines, the rule for BP6 is: "Reputable source reports variant as benign without evidence." The evidence for this variant shows: no such reports. Therefore, this criterion is not applied.

BP7

BP7 (Not Applied)

According to PIK3CA VCEP guidelines, the rule for BP7 is: "Supporting for synonymous/intronic/UTR variants with low conservation." The evidence for this variant shows: it is a missense substitution. Therefore, this criterion is not applied.