About CHEK2
No gene-level summary was recorded for this case.
No variant summary was recorded for this case.
Five-step scale from benign to pathogenic. This variant is uncertain. Select a step to read what it means.
Uncertain.The recorded classification is uncertain significance.
Gene-level background from the recorded interpretation.
Words with a dotted underline have a short definition. Hover or tap them.No gene-level summary was recorded for this case.
The recorded framework is ClinGen CHEK2 VCEP v1.0.0. The criteria and strengths shown below come from the saved assessment; this page does not recalculate the classification.
The applied criteria and their strengths are shown as assessed under the stated framework.
NM_007194.4:c.1513T>A is present at very low frequency in general population databases. gnomAD v2.1: 17/233,642 alleles (AF=0.00728%). gnomAD v4.1: 44/1,595,378 alleles (AF=0.00276%). The highest subpopulation frequency is in East Asians at 0.083% (v2.1, 15/17,996) and 0.078% (v4.1, 35/44,872), both below the 0.1% PM2 threshold for a gene with CHEK2-associated cancer predisposition. No homozygotes observed. Absent from gnomAD-Canada.
Multiple lines of computational evidence unanimously predict no damaging effect for p.Ser505Thr. REVEL score is 0.018 (benign range), BayesDel score is -0.428 (benign range), and SpliceAI predicts no splice alteration (max delta = 0.00). These complementary in silico tools provide consistent evidence that the missense substitution is tolerated at the protein level.
Hereditary Breast, Ovarian and Pancreatic Cancer Specification v1.0.0 lacked a usable explicit final combination framework, so generic ACMG/AMP 2015 final-combination rules were applied as fallback; applied criteria: PM2 supporting, BP4 supporting; combination = 1 supporting + 1 supporting benign, which maps to VUS.
Evidence summaries and captured source records, where available. Missing entries should not be interpreted as negative findings.
gnomAD v4.1: 44 / 1,595,378 alleles. Recorded AF 2.75797e-05. Recorded homozygote count 0.
This variant is present in gnomAD v2.1 (AF= 7.27609e-05; MAF= 0.00728%, 17/233642 alleles, homozygotes = 0) and has highest observed frequency in the East Asian population (AF= 0.000833519; MAF= 0.08335%, 15/17996 alleles, homozygotes = 0); grpmax FAF= 0.00051369.
Recorded SpliceAI maximum delta score: 0.000. Refer to the recorded PP3/BP4 rationale for interpretation.
4 Likely benign · 1 Uncertain significance · 1 likely benign.
OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. CHEK2, an intracellular kinase involved in control of the cell cycle, is altered in various cancer types.
This variant has not previously been reported in somatic cancers (COSMIC).
This variant does not lie in a statistically significant hotspot.
ACMG/AMP criteria under ClinGen CHEK2 VCEP v1.0.0, with the data and sources behind each one.
26 criteria with recorded statuses: 2 met · 15 not met · 3 not assessed · 6 not applicable. Open any row for the full reasoning, the rule and the evidence behind it.
NM_007194.4:c.1513T>A (p.Ser505Thr) is a missense variant in exon 14 of CHEK2, a gene associated with autosomal dominant hereditary breast, ovarian, pancreatic, and prostate cancer predisposition.
This variant is present at very low frequency in gnomAD: 17/233,642 alleles in v2.1 (AF=0.00728%) and 44/1,595,378 alleles in v4.1 (AF=0.00276%), with the highest subpopulation frequency of 0.083% in East Asians, all below the 0.1% PM2 threshold (PM2_Supporting).
Multiple in silico tools unanimously predict a benign effect: REVEL score 0.018 (benign range), BayesDel score -0.428 (benign range), and SpliceAI max delta 0.00 (no predicted splice impact) (BP4_Supporting).
PVS1 is not applicable as this is a missense variant, not a null variant. PS1 and PM5 are not applicable as no same-residue pathogenic comparators have been identified. BP7 is not applicable as the variant is not synonymous.
ClinVar consensus is Likely benign from four clinical laboratories, with one additional submitter reporting Uncertain significance and one reporting likely benign. No expert panel classification is available, and no reputable source has classified this variant as pathogenic.
Functional evidence (PS3/BS3) remains unassessed pending full-text review of PMID:30851065 (Delimitsou et al. 2019), which performed functional characterization of CHEK2 variants in a yeast system and may include p.Ser505Thr.
No de novo reports (PS2/PM6), no case-control enrichment data (PS4), no co-segregation data (PP1), and no trans-observation with a pathogenic variant (BP2) are available for this variant.
Applying generic ACMG/AMP 2015 combination rules (PMID:25741868) with PM2_Supporting (1 pathogenic point) and BP4_Supporting (1 benign point), the variant is classified as a Variant of Uncertain Significance (VUS), with the benign and pathogenic evidence effectively balancing each other. The CSPEC CHEK2 VCEP v1.0.0 (doc 522546466) did not provide machine-interpretable criteria beyond the raw ruleset.
No criteria match.
Every interpreted variant in CHEK2, mapped onto the exon structure of NM_007194.4. Select a row to find it on the plot.
| Protein | Location | Classification | Link |
|---|
| East Asian35 / 44,872 | 0.00078 |
| Remaining individuals7 / 62,170 | 0.00011 |
| Admixed American2 / 59,982 | 3.3e-05 |
| East Asian15 / 17,996 | 0.00083 |
| Admixed American2 / 34,358 | 5.8e-05 |
This variant is present in gnomAD v4.1 (AF= 2.75797e-05; MAF= 0.00276%, 44/1595378 alleles, homozygotes = 0) and has highest observed frequency in the East Asian population (AF= 0.000779996; MAF= 0.07800%, 35/44872 alleles, homozygotes = 0); grpmax FAF= 0.00057582.
This variant is present in gnomAD v2.1 (AF= 7.27609e-05; MAF= 0.00728%, 17/233642 alleles, homozygotes = 0) and has highest observed frequency in the East Asian population (AF= 0.000833519; MAF= 0.08335%, 15/17996 alleles, homozygotes = 0); grpmax FAF= 0.00051369.
SpliceAI predicts no significant splice impact for this variant (max delta score = 0.00). REVEL score = 0.018. BayesDel score = -0.428192.
9 paper records listed. No publication is explicitly linked to an applied criterion in the recorded metadata. None of the listed paper records explicitly indicates that the variant is named.
Screenshots represent source records captured during evidence collection. Select one to view the recorded source content.
The evidence statements recorded by the interpretation pipeline. They have not been reinterpreted by the page.
This variant is present in gnomAD v4.1 (AF= 2.75797e-05; MAF= 0.00276%, 44/1595378 alleles, homozygotes = 0) and has highest observed frequency in the East Asian population (AF= 0.000779996; MAF= 0.07800%, 35/44872 alleles, homozygotes = 0); grpmax FAF= 0.00057582.
This variant is present in gnomAD v2.1 (AF= 7.27609e-05; MAF= 0.00728%, 17/233642 alleles, homozygotes = 0) and has highest observed frequency in the East Asian population (AF= 0.000833519; MAF= 0.08335%, 15/17996 alleles, homozygotes = 0); grpmax FAF= 0.00051369.
This variant has been reported in ClinVar as Likely benign (4 clinical laboratories) and as Uncertain significance (1 clinical laboratory) and as likely benign (1 clinical laboratory). (ClinVarID = 182441)
SpliceAI predicts no significant splice impact for this variant (max delta score = 0.00). REVEL score = 0.018. BayesDel score = -0.428192.
OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. CHEK2, an intracellular kinase involved in control of the cell cycle, is altered in various cancer types.
This variant does not lie in a statistically significant hotspot. This variant has not previously been reported in somatic cancers (COSMIC).
This variant does not lie in a statistically significant hotspot.