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.1220G>T
Protein Change
C407F
Location
Exon 7 (Exon 7 of )
7
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
COSM6941893
Recurrence
5 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 C407F 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
0.558
Threshold: ≥0.75 = PP3 applied

SpliceAI Scores

Window: ±500bp
Effect Type Score Position
- Acceptor Loss (AL) 0.0 -74 bp
- Donor Loss (DL) 0.0 31 bp
+ Acceptor Gain (AG) 0.01 12 bp
+ Donor Gain (DG) 0.0 20 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, frameshift, start codon, splicing +1/2) in a gene where LOF is a known mechanism of disease.' The evidence for this variant shows: it is a missense change (C407F), not a null variant. Therefore, this criterion is not applied.

PS1

PS1 (Not Applied)

According to VCEP guidelines, the rule for PS1 is: '**Strong Strength**: Strong No change.' The evidence for this variant shows: no previously established pathogenic variant results in the same amino acid change at position 407. Therefore, this criterion is not applied.

PS2

PS2 (Not Applied)

According to VCEP guidelines, the rule for PS2 is: 'Strong: Award PS2_Strong if Criteria 1 AND Criteria 2 are fulfilled; Moderate: Award PS2_Moderate if Criteria 1 is fulfilled or if parents are not available but Criteria 2 is fulfilled.' The evidence for this variant shows: no data on de novo occurrence or mosaicism. Therefore, this criterion is not applied.

PS3

PS3 (Not Applied)

According to VCEP guidelines, the rule for PS3 is: 'Strong, Moderate, or Supporting depending on validated functional assay quality metrics.' The evidence for this variant shows: no functional studies have been performed. Therefore, this criterion is not applied.

PS4

PS4 (Not Applied)

According to VCEP guidelines, the rule for PS4 is: 'Points assigned based on phenotypic case data to determine strength; applicable only if variant meets PM2.' The evidence for this variant shows: no reported cases in the literature or patient cohorts. Therefore, this criterion is not applied.

PM1

PM1 (Not Applied)

According to VCEP guidelines, the rule for PM1 is: 'Supporting for residues affecting critical functional domains provided in Table 4 for each gene.' The evidence for this variant shows: position 407 is not within a VCEP-defined hotspot or critical domain for PIK3CA. Therefore, this criterion is not applied.

PM2

PM2 (Supporting)

According to 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: it is absent from gnomAD and other population databases (MAF = 0%). Therefore, this criterion is applied at Supporting strength.

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 disorders are not recessive and no trans observations. 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 indels or stop-loss variants.' 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 VCEP guidelines, the rule for PM5 is: 'Moderate Strength: No change.' The evidence for this variant shows: no other missense change at codon 407 has been established as pathogenic. 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.' 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 (Supporting)

According to VCEP guidelines, the rule for PP2 is: 'Supporting Strength: Missense constraint computed in ExAC/gnomAD (z-score >3.09) applicable to PIK3CA.' The evidence for this variant shows: PIK3CA is missense-constrained (z-score >3.09) and this is a missense change. Therefore, this criterion is applied at Supporting strength.

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 tools yield mixed predictions with no consensus. 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 highly specific for gene.' 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.' The evidence for this variant shows: not found in ClinVar or other databases. Therefore, this criterion is not applied.

BA1

BA1 (Not Applied)

According to VCEP guidelines, the rule for BA1 is: 'Stand Alone strength for allele frequency >0.0926%.' The evidence for this variant shows: allele frequency is 0%. Therefore, this criterion is not applied.

BS1

BS1 (Not Applied)

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

BS2

BS2 (Not Applied)

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

BS3

BS3 (Not Applied)

According to VCEP guidelines, the rule for BS3 is: 'Strong or Supporting for well-validated functional assays showing no damaging effect.' The evidence for this variant shows: no functional assays performed. 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 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 loss-of-function causes disease.' The evidence for this variant shows: PIK3CA gain-of-function missense variants cause disease. Therefore, this criterion is not applied.

BP2

BP2 (Not Applied)

According to standard ACMG guidelines, the rule for BP2 is: 'Observed in cis or trans with a pathogenic variant in the same gene.' The evidence for this variant shows: no 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 indels in repetitive region without known function.' The evidence for this variant shows: it is a missense variant, not an indel. Therefore, this criterion is not applied.

BP4

BP4 (Not Applied)

According to VCEP guidelines, the rule for BP4 is: 'Only for synonymous, intronic, or UTR variants if two of three splicing tools predict no impact.' The evidence for this variant shows: it is a missense change. 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 such data. 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.' The evidence for this variant shows: no such reports. Therefore, this criterion is not applied.

BP7

BP7 (Not Applied)

According to VCEP guidelines, the rule for BP7 is: 'For synonymous, intronic, or UTR variants with non-conserved nucleotide.' The evidence for this variant shows: it is a missense change. Therefore, this criterion is not applied.