About MSH3
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 likely benign. Select a step to read what it means.
Likely benign.A likely benign classification under the recorded framework.
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 Not recorded. 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.
This variant is extremely rare in large population databases: gnomAD v2.1 AF=4.32×10⁻⁶ (1/231,498 alleles), gnomAD v4.1 AF=3.13×10⁻⁶ (5/1,599,926 alleles), grpmax FAF=1.25×10⁻⁶, and absent from gnomAD-Canada. All observed allele frequencies are well below the 0.1% PM2 threshold. No homozygous individuals have been observed. One ClinVar submitter (Labcorp/Invitae) noted potential data quality concerns at this position in gnomAD but did not provide alternative frequency data.
This is a missense variant (p.Arg3His) in MSH3, a gene for which the established disease mechanism is primarily biallelic loss-of-function via truncating variants. MSH3-related autosomal recessive adenomatous polyposis is caused by biallelic LoF variants. A missense variant in a gene where primarily truncating variants are known to cause disease meets BP1.
Multiple lines of computational evidence suggest no impact on the gene product. REVEL score is 0.31 (below pathogenic threshold of 0.5). BayesDel score is -0.0159 (not damaging). SpliceAI predicts no splicing impact (max delta 0.00). The concordant benign predictions from multiple algorithm types support BP4.
gene-specific framework lacked a usable explicit final combination framework, so generic ACMG/AMP 2015 final-combination rules were applied as fallback; applied criteria: PM2 moderate, BP1 supporting benign, BP4 supporting benign; combination = 1 moderate + 2 supporting benign, which maps to Likely Benign.
Evidence summaries and captured source records, where available. Missing entries should not be interpreted as negative findings.
gnomAD v4.1: 5 / 1,599,926 alleles. Recorded AF 3.12514e-06. Recorded homozygote count 0.
This variant is present in gnomAD v2.1 (AF= 4.31969e-06; MAF= 0.00043%, 1/231498 alleles, homozygotes = 0) and has highest observed frequency in the Admixed American population (AF= 3.02737e-05; MAF= 0.00303%, 1/33032 alleles, homozygotes = 0).
Absent from gnomAD-Canada v1.0.
Recorded SpliceAI maximum delta score: 0.000. Refer to the recorded PP3/BP4 rationale for interpretation.
2 Uncertain significance. ClinVar API review status: criteria provided, multiple submitters, no conflicts.
OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. MSH3, a DNA mismatch repair protein, is frequently mutated in colorectal cancers.
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 Not recorded, with the data and sources behind each one.
29 criteria with recorded statuses: 3 met · 19 not met · 2 not assessed · 5 not applicable. Open any row for the full reasoning, the rule and the evidence behind it.
NM_002439.5:c.8G>A (p.Arg3His) in MSH3 is a missense variant in exon 1. This variant is extremely rare in population databases (gnomAD v4.1 AF=3.13×10⁻⁶, 5/1,599,926 alleles; gnomAD v2.1 AF=4.32×10⁻⁶, 1/231,498 alleles; 0 homozygotes observed).
The variant has been reported in ClinVar as Uncertain significance by two clinical laboratories (ClinVar ID: 969087). No expert panel classification is available. The ClinVar-associated references (PMID:25394175, a cancer referral practice guideline, and PMID:28492532, the Sherloc classification framework paper) do not provide variant-specific evidence for NM_002439.5:c.8G>A.
Computational predictors do not support a deleterious effect: REVEL score 0.31 (below pathogenic threshold), BayesDel score -0.0159 (not damaging), and SpliceAI max delta 0.00 (no splicing impact predicted).
No functional studies, segregation data, de novo reports, or case-control observations have been published for this variant. OncoKB reports Unknown Oncogenic Effect with no variant-specific functional evidence.
Evidence weighting: one moderate pathogenic criterion (PM2 — extremely low population frequency) and two supporting benign criteria (BP1 — missense in a primarily loss-of-function disease gene; BP4 — multiple concordant benign computational predictions). The net evidence is insufficient to classify this variant as pathogenic or benign, consistent with a variant of uncertain significance.
No criteria match.
Every interpreted variant in MSH3, mapped onto the exon structure of NM_002439.5. Select a row to find it on the plot.
| Protein | Location | Classification | Link |
|---|
| European (non-Finnish)5 / 1,175,206 | 4.3e-06 |
| Admixed American1 / 33,032 | 3e-05 |
This variant is present in gnomAD v4.1 (AF= 3.12514e-06; MAF= 0.00031%, 5/1599926 alleles, homozygotes = 0) and has highest observed frequency in the European (non-Finnish) population (AF= 4.25457e-06; MAF= 0.00043%, 5/1175206 alleles, homozygotes = 0); grpmax FAF= 1.25e-06.
This variant is present in gnomAD v2.1 (AF= 4.31969e-06; MAF= 0.00043%, 1/231498 alleles, homozygotes = 0) and has highest observed frequency in the Admixed American population (AF= 3.02737e-05; MAF= 0.00303%, 1/33032 alleles, homozygotes = 0).
Absent from gnomAD-Canada v1.0.
SpliceAI predicts no significant splice impact for this variant (max delta score = 0.00). REVEL score = 0.31. BayesDel score = -0.015906.
1 paper record 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= 3.12514e-06; MAF= 0.00031%, 5/1599926 alleles, homozygotes = 0) and has highest observed frequency in the European (non-Finnish) population (AF= 4.25457e-06; MAF= 0.00043%, 5/1175206 alleles, homozygotes = 0); grpmax FAF= 1.25e-06.
This variant is present in gnomAD v2.1 (AF= 4.31969e-06; MAF= 0.00043%, 1/231498 alleles, homozygotes = 0) and has highest observed frequency in the Admixed American population (AF= 3.02737e-05; MAF= 0.00303%, 1/33032 alleles, homozygotes = 0).
Absent from gnomAD-Canada v1.0.
This variant has been reported in ClinVar as Uncertain significance (2 clinical laboratories). (ClinVarID = 969087)
SpliceAI predicts no significant splice impact for this variant (max delta score = 0.00). REVEL score = 0.31. BayesDel score = -0.015906.
OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. MSH3, a DNA mismatch repair protein, is frequently mutated in colorectal cancers.
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.