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.1A>G
Protein Change
M1?
Location
Exon 2 (Exon 2 of )
2
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
Uncertain Significance (VUS)
1 publications
Clinical Statement

"This variant has been reported in ClinVar as Uncertain significance (4 clinical laboratories)."

COSMIC Somatic Evidence

Open
COSMIC ID
COSM1041442
Recurrence
8 occurrences
PM1 Criteria
Not Applied

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 M1V variant has conflicting functional evidence. In a breast cell line, it demonstrated an activating effect by increasing growth-factor independent proliferation. However, in a murine B-cell line and a transgenic mouse model, it showed a neutral effect, failing to promote growth-factor independent proliferation or glioblastoma formation.

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
0.0
Threshold: ≥0.75 = PP3 applied

SpliceAI Scores

Window: ±500bp
Effect Type Score Position
- Acceptor Loss (AL) 0.0 22 bp
- Donor Loss (DL) 0.02 209 bp
+ Acceptor Gain (AG) 0.0 215 bp
+ Donor Gain (DG) 0.01 21 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, canonical ±1 or 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: c.1A>G is a start codon change in PIK3CA, a gene whose disease mechanism is gain-of-function rather than loss-of-function. Therefore, this criterion is not applied.

PS1

PS1 (Not Applied)

According to standard ACMG guidelines, the rule for PS1 is: '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 codon 1 (M1). Therefore, this criterion is not applied.

PS2

PS2 (Not Applied)

According to standard ACMG guidelines, the rule for PS2 is: 'De novo (both maternity and paternity confirmed) in a patient with the disease and no family history.' The evidence for this variant shows: no data on de novo occurrence. Therefore, this criterion is not applied.

PS3

PS3 (Not Applied)

According to VCEP guidelines, the rule for PS3 is: 'Award PS3 if the functional assay meets the acceptability criteria with quality metrics and minimum validation controls (animal models considered separately).' The evidence for this variant shows: functional studies are conflicting and do not meet VCEP assay criteria. Therefore, this criterion is not applied.

PS4

PS4 (Not Applied)

According to VCEP guidelines, the rule for PS4 is: 'Points are assigned for phenotype with absent controls; strength based on total points.' The evidence for this variant shows: no case‐level phenotype data reported. Therefore, this criterion is not applied.

PM1

PM1 (Not Applied)

According to VCEP guidelines, the rule for PM1 is: 'Supporting: residues affecting critical functional domains provided in Table 4 for each gene.' The evidence for this variant shows: codon 1 is not within a defined critical functional domain of 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.' The evidence for this variant shows: absent from gnomAD and other population databases. Therefore, this criterion is applied at Supporting strength.

PM3

PM3 (Not Applied)

According to standard ACMG guidelines, the rule for PM3 is: 'For recessive disorders, detected in trans with a pathogenic variant.' The evidence for this variant shows: PIK3CA disease is not recessive and no trans data are available. Therefore, this criterion is not applied.

PM4

PM4 (Not Applied)

According to standard ACMG guidelines, the rule for PM4 is: 'Protein length changes as a result of in‐frame deletions/insertions or stop‐loss.' The evidence for this variant shows: c.1A>G is a start codon loss, not an in‐frame indel or stop‐loss. Therefore, this criterion is not applied.

PM5

PM5 (Not Applied)

According to standard ACMG guidelines, the rule for PM5 is: 'Novel missense change at an amino acid residue where a different missense change is pathogenic.' The evidence for this variant shows: no pathogenic missense changes reported at Met1. 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 and 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 family segregation data. Therefore, this criterion is not applied.

PP2

PP2 (Supporting)

According to VCEP guidelines, the rule for PP2 is: 'Supporting: missense constraint computed in ExAC/gnomAD; award PP2 if z‐score >3.09.' The evidence for this variant shows: PIK3CA missense constraint z‐score in gnomAD is >3.09. 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: mixed in silico predictions with a majority suggesting no 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 specific phenotype data. Therefore, this criterion is not applied.

PP5

PP5 (Not Applied)

According to standard ACMG guidelines, the rule for PP5 is: 'Reputable source recently reports variant as pathogenic.' The evidence for this variant shows: ClinVar classifies as VUS by multiple submitters. Therefore, this criterion is not applied.

BA1

BA1 (Not Applied)

According to standard ACMG guidelines, the rule for BA1 is: 'Allele frequency >5% in control populations.' The evidence for this variant shows: allele frequency is 0% in gnomAD. Therefore, this criterion is not applied.

BS1

BS1 (Not Applied)

According to VCEP guidelines, the rule for BS1 is: 'Strong: allele frequency >0.0185% in control databases.' 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: observed in ≥3 healthy individuals (homozygous or heterozygous).' The evidence for this variant shows: no observations in well‐phenotyped adults. Therefore, this criterion is not applied.

BS3

BS3 (Not Applied)

According to VCEP guidelines, the rule for BS3 is: 'Strong/Supporting: well‐established functional studies show no damaging effect.' The evidence for this variant shows: conflicting functional results not meeting VCEP quality criteria. 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 a gene for which primarily truncating variants cause disease.' The evidence for this variant shows: PIK3CA disease mechanism is gain‐of‐function missense. Therefore, this criterion is not applied.

BP2

BP2 (Not Applied)

According to standard ACMG guidelines, the rule for BP2 is: 'Observed in cis with a pathogenic variant.' The evidence for this variant shows: no such 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 regions.' The evidence for this variant shows: not an indel or repetitive region. Therefore, this criterion is not applied.

BP4

BP4 (Not Applied)

According to VCEP guidelines, the rule for BP4 is: 'Supporting for synonymous/intronic variants if two of three splicing tools predict no impact.' The evidence for this variant shows: not a synonymous or intronic variant. 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.' The evidence for this variant shows: no such cases 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.' 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: 'Supporting for synonymous/intronic variants with low conservation.' The evidence for this variant shows: not a synonymous or intronic variant. Therefore, this criterion is not applied.