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Print report
Variant interpretation report

MLH1 c.232G>C, p.Glu78Gln

NM_000249.4:c.232G>C NP_000240.1:p.(Glu78Gln) Missense · exon 3 View identifiers
Classification
Uncertain significance

Variant description:

The MLH1 NM_000249.4:c.232G>C (NP_000240.1:p.(Glu78Gln)) missense variant has not previously been reported in somatic cancers (COSMIC). Based on available guidelines, this variant is considered to be a Variant of Uncertain Significance (VUS).

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.

In plain language

Gene-level background from the recorded interpretation.

About MLH1

MLH1 encodes a DNA mismatch repair protein that, together with partners such as PMS2, corrects errors that arise during DNA replication. Loss of this repair function leads to the accumulation of mutations, particularly in repetitive microsatellite sequences, and tumors with defective MLH1 typically show high microsatellite instability. Germline mutations in MLH1 cause Lynch syndrome (hereditary non-polyposis colorectal cancer), which strongly predisposes to colorectal, endometrial, ovarian, and other cancers, while biallelic mutations cause constitutional mismatch repair deficiency (CMMRD). Because it normally protects against cancer by maintaining genomic stability, MLH1 acts as a tumor suppressor, and mismatch-repair-deficient tumors often respond well to immunotherapy.

LYFE Sciences is an AI system, and it can make mistakes. Criteria may be applied incorrectly, sources may be misread, and a confident-looking classification can still be wrong. Double-check every criterion and its underlying evidence before relying on any call.
01The reasoning

How it was classified

The recorded framework is ClinGen MLH1 VCEP v2.0. The criteria and strengths shown below come from the saved assessment; this page does not recalculate the classification.

Recorded applied criteria

The applied criteria and their strengths are shown as assessed under the stated framework.

Pathogenic evidence criteria: 2 Benign evidence criteria: 0
Pathogenic criteria applied · 2
PM2Supporting
PM2 · supporting

Met at supporting: gnomAD v4 total AF is 1.2441e-06, below the VCEP PM2 threshold of 0.00002 (1 in 50,000 alleles).

PP3Supporting
PP3 · supporting

Met at Supporting: HCI prior probability is 0.7144, within the VCEP PP3 Supporting range (>0.68 and ≤0.81).

Benign criteria applied · 0

No benign criterion marked as applied in the recorded assessment.

Recorded classification: Uncertain significance

VUS: PM2 (supporting) and PP3 (supporting) are met, but the MLH1 VCEP's criteria-combination rules do not yield a pathogenic or benign classification.

02The sources

Evidence at a glance

Evidence summaries and captured source records, where available. Missing entries should not be interpreted as negative findings.

No screenshot for this evidence
REVEL score
0.855 recorded score

Recorded REVEL score: 0.855. Refer to the recorded PP3/BP4 rationale for interpretation.

ClinVar submissions
1 reported laboratory submissions

1 Uncertain significance. ClinVar API review status: criteria provided, single submitter.

Context only ClinVar ↗
Functional evidence
Statement recorded

OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. MLH1, a DNA mismatch repair protein, is recurrently altered by deletion and mutation in various cancer types.

No listed criterion applied OncoKB ↗
COSMIC
Cancer Hotspots
Somatic evidence
No matching record reported

This variant has not previously been reported in somatic cancers (COSMIC).

Cancer Hotspots

This variant does not lie in a statistically significant hotspot.

For specialists

Technical detail

ACMG/AMP criteria under ClinGen MLH1 VCEP v2.0, with the data and sources behind each one.

28 criteria with recorded statuses: 2 met · 3 not met · 11 not assessed · 12 not applicable. Open any row for the full reasoning, the rule and the evidence behind it.

Met Not met Not assessed Not applicable

All three requested criteria are not applicable under the MLH1 VCEP CSPEC v2.0 entries: PVS1, PM4, and BP3 are each explicitly marked Not Applicable for this missense variant.

PS4, PP5, and BP6 are not applicable under the governing MLH1 VCEP.

PP4 and BP5 remain not assessed because the case lacks the patient and tumor findings required by their VCEP-specific rules.

PMID:25394175 was reviewed in full as the only indexed PS4/PP5 lead; it does not mention this variant or provide variant-specific evidence for these criteria.

Population evidence supports PM2 at Supporting strength because gnomAD v4 total AF and grpmax FAF are below 0.00002.

BA1 and BS1 are not met because the gnomAD v4 grpmax FAF is below both VCEP benign frequency thresholds.

BS2 is not assessed because no qualifying individual-level in-trans co-occurrence and phase evidence is available.

Met · 2

PM2 SupportingVCEPMet at supporting: gnomAD v4 total AF is 1.2441e-06, below the VCEP PM2 threshold of 0.00002 (1 in 50,000 alleles). Details ▾Hide ▴
Reasoning

PM2_Supporting is met. The governing MLH1 VCEP rule requires an absent or extremely rare allele frequency below 0.00002 (less than 1 in 50,000 alleles) in gnomAD v4. The variant is present at total AF 1.2441e-06 (2/1,607,590 alleles), grpmax FAF 3.65e-06, and zero homozygotes, all below 0.00002; the VCEP pilot-variants table was searched under c.232G>C, p.Glu78Gln, and p.E78Q with no matching entry.

VCEP rule
Absent/extremely rare allele frequency \<0.00002 (\<1 in 50,000 alleles ) in gnomAD v4 dataset
Evidence considered
  • gnomAD v4.1 reports total AF 1.2441e-06 (2/1,607,590 alleles), exome AF 1.374220988477157e-06 (2/1,455,370 alleles), grpmax FAF 3.65e-06, and homozygotes = 0.
  • The governing rule requires: Absent/extremely rare allele frequency <0.00002 (<1 in 50,000 alleles) in gnomAD v4 dataset.
  • VCEP-pilot-variants---MMR.xlsx, full-text conversion: no matching row for c.232G>C, p.Glu78Gln, or p.E78Q.
Still missing
  • VCEP-pilot-variants---MMR.xlsx was searched for c.232G>C, p.Glu78Gln, and p.E78Q; no entry for this variant was found.
Sources
PP3 SupportingVCEPMet at Supporting: HCI prior probability is 0.7144, within the VCEP PP3 Supporting range (>0.68 and ≤0.81). Details ▾Hide ▴
Reasoning

The variant is a missense substitution, so the VCEP's missense HCI-prior pathway applies. The governing HCI-PRIORS-MLH1 table contains the exact entry c.232G>C / p.E78Q with prior probability 0.7144. This is greater than 0.68 and no greater than 0.81, satisfying PP3 at Supporting strength. The table's pre-assigned result governs; REVEL, BayesDel, and the SpliceAI no-impact score are not used for this PP3 decision.

VCEP rule
Missense variant with HCI prior probability for pathogenicity >0.68 & ≤0.81 as per [https://hci-priors.hci.utah.edu/PRIORS](https://hci-priors.hci.utah.edu/PRIORS) OR Predicted splice defect for non-canonical splicing nucleotides using SpliceAI with delta score >= 0.2 as per Walker et al 2023.
Evidence considered
  • HCI-PRIORS-MLH1.txt.txt, exact row for c.232G>C / p.E78Q: prior probability of pathogenicity = 0.7144, MAPP score = 13.2, PMID22949387 listed in the row.
  • The VCEP CSPEC identifies missense HCI prior probability >0.68 and ≤0.81 as PP3 Supporting; the exact table entry is therefore applied directly.
Sources

Not met · 3 · evaluated, threshold not reached

BA1 VCEPNot met: gnomAD v4 grpmax FAF is 3.65e-06, below the VCEP BA1 threshold of 0.001 (0.1%). Details ▾Hide ▴
Reasoning

BA1 is not met. The governing MLH1 VCEP rule requires a gnomAD v4 Grpmax filtering allele frequency of at least 0.001 (0.1%) and exclusion as a founder pathogenic variant. The observed gnomAD v4 grpmax FAF is 3.65e-06, far below 0.001; the VCEP pilot-variants table was searched under c.232G>C, p.Glu78Gln, and p.E78Q with no matching entry.

VCEP rule
GnomAD v4 Grpmax filtering allele frequency ≥ 0.001 (0.1%) and variant is excluded as founder pathogenic variant.
Evidence considered
  • gnomAD v4.1 reports total AF 1.2441e-06 (2/1,607,590 alleles), highest South Asian AF 2.19949e-05 (2/90,930 alleles), and grpmax FAF 3.65e-06; homozygotes = 0.
  • The governing rule requires: GnomAD v4 Grpmax filtering allele frequency ≥ 0.001 (0.1%) and variant is excluded as founder pathogenic variant.
  • VCEP-pilot-variants---MMR.xlsx, full-text conversion: no matching row for c.232G>C, p.Glu78Gln, or p.E78Q.
Still missing
  • VCEP-pilot-variants---MMR.xlsx was searched for c.232G>C, p.Glu78Gln, and p.E78Q; no entry for this variant was found.
Sources
BS1 VCEPNot met: gnomAD v4 grpmax FAF is 3.65e-06, below the VCEP BS1 lower threshold of 0.0001 (0.01%). Details ▾Hide ▴
Reasoning

BS1 is not met. The governing MLH1 VCEP rule requires a gnomAD v4 Grpmax filtering allele frequency of at least 0.0001 and less than 0.001 (0.01–0.1%), with exclusion as a founder pathogenic variant. The observed gnomAD v4 grpmax FAF is 3.65e-06, below 0.0001; the VCEP pilot-variants table was searched under c.232G>C, p.Glu78Gln, and p.E78Q with no matching entry.

VCEP rule
GnomAD v4 Grpmax filtering allele frequency ≥ 0.0001 and \< 0.001 (0.01-0.1%) and variant is excluded as founder pathogenic variant.
Evidence considered
  • gnomAD v4.1 reports grpmax FAF 3.65e-06, with total AF 1.2441e-06 (2/1,607,590 alleles) and no homozygotes.
  • The governing rule requires: GnomAD v4 Grpmax filtering allele frequency ≥ 0.0001 and < 0.001 (0.01-0.1%) and variant is excluded as founder pathogenic variant.
  • VCEP-pilot-variants---MMR.xlsx, full-text conversion: no matching row for c.232G>C, p.Glu78Gln, or p.E78Q.
Still missing
  • VCEP-pilot-variants---MMR.xlsx was searched for c.232G>C, p.Glu78Gln, and p.E78Q; no entry for this variant was found.
Sources
BP4 VCEPNot met: HCI prior probability is 0.7144, not below the VCEP BP4 cutoff of 0.11 for missense variants. Details ▾Hide ▴
Reasoning

The variant is missense, so the VCEP's HCI-prior pathway is the applicable BP4 path. The exact HCI-PRIORS-MLH1 entry for c.232G>C / p.E78Q gives a pathogenicity prior of 0.7144, not less than 0.11; therefore BP4 Supporting is not met. The VCEP's alternative SpliceAI no-impact path is explicitly limited to intronic and synonymous variants and is not applicable to this missense variant.

VCEP rule
Missense variant with HCI-prior probability of pathogenicity \<0.11 as per [https://hci-priors.hci.utah.edu/PRIORS](https://hci-priors.hci.utah.edu/PRIORS) OR For intronic and synonymous variants: SpliceAI predicts no splicing impact with delta score \<= 0.1 as per Walker et al 2023
Evidence considered
  • HCI-PRIORS-MLH1.txt.txt, exact row for c.232G>C / p.E78Q: prior probability of pathogenicity = 0.7144.
  • SpliceAI max delta score = 0.04 is present in the case evidence, but the VCEP BP4 SpliceAI alternative applies only to intronic and synonymous variants, not this missense substitution.
Sources

Not assessed · 11 · no assessment recorded

PS1 VCEPNot assessed: no VCEP-established Pathogenic variant with a different nucleotide change encoding p.Glu78Gln was identified. Details ▾Hide ▴
Reasoning

The variant is a missense substitution, but the available VCEP-governed evidence does not identify a different nucleotide change encoding p.Glu78Gln/p.E78Q that this VCEP previously established as Pathogenic, nor a qualifying same-nucleotide splice comparison. The PM5 comparator collection found zero same-residue candidates and the ClinVar record is a single-submitter uncertain-significance record, so PS1 cannot be evaluated under the VCEP rule.

VCEP rule
A predicted missense substitution that encodes the same amino acid change with a different underlying nucleotide change previously established by this VCEP as Pathogenic (not a predicted or confirmed splice defect).
Evidence considered
  • The target is NM_000249.4:c.232G>C, encoding NP_000240.1:p.(Glu78Gln)/p.(E78Q).
  • The PM5 comparator collection reported total_candidates_seen=9 but same_residue_candidates=0 and expert_panel_plp_candidates=0; it identified no useful same-residue comparator.
  • ClinVar variation 1789704 has one single-submitter Uncertain significance submission and no expert-panel submission, so it does not establish a qualifying VCEP Pathogenic comparator.
Still missing
  • A VCEP-established Pathogenic comparator encoding p.Glu78Gln/p.E78Q through a different nucleotide change is not available in the collected evidence.
Sources
cspec ↗pm5_candidatesclinvar ↗
PS2 VCEPNot assessed: no documented de novo observation, parental testing, or de novo point total is available for this variant. Details ▾Hide ▴
Reasoning

The VCEP provides PS2 point-based de novo evidence thresholds, but the available case evidence contains no documented proband-level de novo observation, parental testing results, confirmed maternity/paternity, or calculable de novo points for NM_000249.4:c.232G>C (p.Glu78Gln). Therefore PS2 cannot be assessed without substituting an unstated rule or assuming de novo status.

VCEP rule
≥ 4 _de novo_ points; 2-3.5 _de novo_ points; 1-1.5 _de novo_ points; 0.5 _de novo_ points
Evidence considered
  • No proband-level de novo observation, parental testing result, or de novo point calculation is documented in the available case evidence for NM_000249.4:c.232G>C (p.Glu78Gln).
Still missing
  • Documented proband-level de novo status
  • Parental genotypes and confirmation of maternity/paternity
  • VCEP-compatible de novo point calculation
Sources
PS3 VCEPNot assessed: no variant-specific functional readout, functional odds, or qualifying RNA result is available to compare with the VCEP PS3 thresholds. Details ▾Hide ▴
Reasoning

PS3 cannot be assessed because the case materials contain no variant-specific functional assay result, functional odds, quantitative repair or expression readout, or qualifying RNA assay for MLH1 c.232G>C (p.Glu78Gln/p.E78Q). The governing VCEP rule permits PS3 Strong for calibrated functional odds >18.7, PS3 Moderate for calibrated functional odds >4.3 and <=18.7, an MMR function defect following the functional assay flowchart, or complete monoallelic expression loss <10% of wild-type with and without NMD block, and PS3 Supporting for calibrated functional odds >2.08 and <=4.3; none can be evaluated from the available evidence. The required VCEP assay documentation and flowchart were searched for c.232G>C, p.Glu78Gln, and p.E78Q, but neither contains a variant-specific entry. The only fetched paper, PMID:25394175, does not mention the variant and provides no functional assay evidence.

VCEP rule
Calibrated functional assays with functional odds for pathogenicity > 18.7 ... Calibrated functional assays with functional odds for pathogenicity >4.3 and <= 18.7 ... OR MMR function defect following functional assay flowchart* ... OR Variants with monoallelic expression: complete loss of expression (<10% of wild-type in cDNA without puromycin) of the variant allele. Full-length transcript should be analysed with and without NMD block. ... Calibrated functional odds for pathogenicity >2.08 and <= 4.3
Evidence considered
  • CSPEC PS3 entries: calibrated functional odds >18.7 support Strong; >4.3 and <=18.7, an MMR function defect following the functional assay flowchart, or complete monoallelic expression loss <10% of wild-type support Moderate; calibrated functional odds >2.08 and <=4.3 support Supporting.
  • Functional-assay-SVI-documentation-MMR.xlsx, sheet MMR assays: validated MLH1 assay rows and their control/threshold framework are listed, but no row identifies c.232G>C, p.Glu78Gln, or p.E78Q and no variant readout is provided.
  • Functional-assay-flowchart.pdf: the missense pathway requires a calibrated functional assay or two independent concordant assays, but no variant-specific assay result is available for this case.
  • Searches of the two governing VCEP files for c.232G>C, p.Glu78Gln, and p.E78Q returned no variant-specific entry.
  • PMID:25394175 paper_extracts entry: the paper does not mention MLH1 c.232G>C or p.(E78Q); its quoted content concerns constitutional mismatch repair deficiency generally, not this variant or a functional assay.
Still missing
  • No variant-specific calibrated functional assay result or functional odds for MLH1 c.232G>C are available.
  • No variant-specific quantitative MMR repair, protein-expression, localization, cellular-activity, or qualifying RNA assay result is available.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in Functional-assay-SVI-documentation-MMR.xlsx, sheet MMR assays.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in Functional-assay-flowchart.pdf.
  • PMID:25394175 was reviewed; its paper extract states that the variant is not mentioned and supplies no variant-specific functional evidence.
Sources
PM3 VCEPNot assessed: gnomAD grpmax FAF is 3.65e-06, but no affected-proband second MLH1 variant or phase information is available to assign PM3 points. Details ▾Hide ▴
Reasoning

The VCEP requires co-occurrence with a known pathogenic or likely pathogenic MLH1 variant in a patient with clinical features consistent with CMMRD, and requires this variant to meet PM2_Supporting. The available evidence establishes the PM2_Supporting prerequisite because gnomAD v4.1 reports a grpmax FAF of 3.65e-06, below the VCEP threshold of 0.00002, but provides no affected-proband observation, second MLH1 variant, phase, or inheritance information. Therefore no PM3 points can be assigned and PM3 cannot be assessed.

VCEP rule
Co-occurrence with a known pathogenic/likely pathogenic sequence variant in the same gene in a patient with clinical features consistent with CMMRD as per Aronson et al 2022 - Refer to “Table for CMMRD diagnosis.pdf”. For MLH1 variants - the variant has to meet PM2\_Supporting criteria. ... Pathogenic/Likely Pathogenic _in trans_: 1.0 point; ... Pathogenic/Likely Pathogenic - phase unknown: 0.5 points ... Sum all cases with the above evidence to determine the PM3 strength.
Evidence considered
  • The VCEP PM3 rule requires co-occurrence with a known pathogenic/likely pathogenic sequence variant in the same gene in a patient with clinical features consistent with CMMRD and requires the MLH1 variant to meet PM2_Supporting.
  • gnomAD v4.1 reports grpmax FAF=3.65e-06 for NM_000249.4:c.232G>C, below the VCEP PM2 threshold of <0.00002; no affected proband, second MLH1 variant, phase, or inheritance observation is reported in the available case evidence.
Still missing
  • Affected-proband evidence with clinical features consistent with CMMRD is unavailable.
  • A known pathogenic or likely pathogenic second MLH1 variant is not documented.
  • Phase or inheritance information establishing trans or phase-unknown status is unavailable.
Sources
PM5 VCEPNot assessed: zero same-residue missense comparator candidates were identified for the required VCEP Pathogenic or Likely Pathogenic comparison. Details ▾Hide ▴
Reasoning

No different missense variant at MLH1 residue 78 classified by this VCEP as Pathogenic or Likely Pathogenic on the protein level and not due to aberrant splicing was identified. The comparator collection reported zero same-residue candidates, so neither the Moderate nor Supporting PM5 path can be applied. The VCEP rule also requires PP3 supporting for PM5; although the case contains an HCI prior of 0.7144 consistent with the VCEP PP3-supporting interval, the required same-residue comparator is absent.

VCEP rule
Missense change at an amino acid residue where a different missense change was classified by this VCEP as Pathogenic on the protein level and not due to aberrant splicing. Only use PM5 if PP3 is supporting for the missense change. Use PM5_Supporting if other variant is Likely Pathogenic due to a missense alteration.
Evidence considered
  • The PM5 comparator collection reported same_residue_candidates=0, expert_panel_plp_candidates=0, and non_expert_majority_plp_candidates=0.
  • The HCI-PRIORS-MLH1 table contains the target row c.232G>C/p.E78Q with prior probability 0.7144; this lies in the VCEP PP3-supporting interval (>0.68 and <=0.81), but it does not supply the required alternate same-residue comparator.
Still missing
  • A different missense variant at residue 78 with a qualifying VCEP Pathogenic or Likely Pathogenic protein-level classification is not available in the collected evidence.
Sources
PP1 VCEPNot assessed: no pedigree data, informative meioses, or combined Bayes Likelihood Ratio is available for co-segregation evaluation. Details ▾Hide ▴
Reasoning

The VCEP requires co-segregation evidence evaluated by a combined Bayes Likelihood Ratio, with supporting, moderate, or strong thresholds. The available case evidence contains no disease pedigree, affected or unaffected relative genotypes and phenotypes, meioses, or combined Bayes Likelihood Ratio for NM_000249.4:c.232G>C (p.Glu78Gln). PP1 therefore cannot be assessed.

VCEP rule
Co-segregation with disease in pedigree(s) with a combined* Bayes Likelihood Ratio >18.7 in ≥2 families; Co-segregation with disease in pedigree(s) with a combined* Bayes Likelihood Ratio >4.3 & ≤18.7; Co-segregation with disease in pedigree(s) with a combined* Bayes Likelihood Ratio >2.08 & ≤4.3
Evidence considered
  • No disease pedigree, relative genotype/phenotype segregation data, informative meioses, or combined Bayes Likelihood Ratio is documented in the available case evidence for NM_000249.4:c.232G>C (p.Glu78Gln).
Still missing
  • Disease pedigree and phenotype data
  • Genotypes of informative affected and unaffected relatives
  • Informative meioses and combined Bayes Likelihood Ratio
Sources
PP4 VCEPNot assessed: no patient tumor MSI/IHC findings or MLH1-methylation exclusion are available to evaluate the PP4 Supporting requirement. Details ▾Hide ▴
Reasoning

PP4 is applicable, but the available case evidence contains no patient or family phenotype, tumor MSI/IHC results, tumor counts, or MLH1 promoter-methylation results. The governing VCEP requires at least one CRC or endometrial MSI-H tumor or loss of MMR protein expression consistent with the variant location, with MLH1 promoter methylation excluded for MLH1 variants. The Table-for-CMMRD-diagnosis.pdf was searched for c.232G>C, p.Glu78Gln, and p.E78Q and had no variant-specific entry; its general CMMRD scoring table cannot supply missing case phenotype. VCEP-pilot-variants---MMR.xlsx.txt was likewise searched and had no entry for this variant.

VCEP rule
1 CRC/Endometrial MSI-H tumor using a standard panel of 5-10 markers<sup>e </sup> or tumor genome **and/or**​ loss of MMR protein expression consistent with the variant location. MSI-H tumor with inconsistent protein expression does not meet PP4. For _MLH1_ variants, _MLH1_ promoter methylation is to be excluded in the tumor.
Evidence considered
  • The VCEP PP4 Supporting rule requires 1 CRC or endometrial MSI-H tumor using a standard 5-10-marker panel or tumor genome and/or loss of MMR protein expression consistent with the variant location, with MLH1 promoter methylation excluded.
  • The case evidence provides no patient phenotype, tumor MSI/IHC result, tumor count, or MLH1 promoter-methylation result.
  • Table-for-CMMRD-diagnosis.pdf, including its CMMRD scoring rows, was searched for c.232G>C, p.Glu78Gln, and p.E78Q; no variant-specific row was found.
  • VCEP-pilot-variants---MMR.xlsx, including its pilot classification rows, was searched for c.232G>C, p.Glu78Gln, and p.E78Q; no variant-specific row was found.
Still missing
  • Patient or family phenotype and tumor records are missing, including MSI status, IHC protein expression, tumor number, and MLH1 promoter-methylation results.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in Table-for-CMMRD-diagnosis.pdf.txt.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in VCEP-pilot-variants---MMR.xlsx.txt.
Sources
BS2 VCEPNot assessed: no individual-level in-trans co-occurrence, confirmed phase, age, and CMMRD information is available for the BS2 rule. Details ▾Hide ▴
Reasoning

BS2 cannot be assessed from the available evidence. The governing MLH1 VCEP rule requires confirmed co-occurrence in trans with a known pathogenic sequence variant in the same gene in a suitably aged patient and no CMMRD manifestations, with phase confirmed through parents or offspring. gnomAD reports zero homozygotes but does not provide the required individual-level co-occurrence, phase, clinical age, or CMMRD information; the VCEP pilot-variants table was searched under c.232G>C, p.Glu78Gln, and p.E78Q with no matching entry.

VCEP rule
Co-occurrence ​_in trans​_ with a known pathogenic sequence variant in the same gene in a patient with colorectal cancer after age 45 (or other LS cancer above the median age of onset for that cancer in LS<sup>​d</sup>), and who has no previous or current evidence of clinical manifestations of CMMRD as per Aronson et al 2022 (Refer to 'Table for CMMRD diagnosis.pdf'). Confirmation of phase requires testing of parents or offspring.
Evidence considered
  • The governing rule requires co-occurrence in trans with a known pathogenic sequence variant in the same gene in a patient with colorectal cancer after age 45 (or another Lynch syndrome cancer above its median age of onset), no previous or current evidence of CMMRD, and parental or offspring testing to confirm phase.
  • gnomAD v4.1 reports homozygotes = 0, but this population aggregate does not establish the individual-level co-occurrence, phase, clinical age, or CMMRD conditions required by BS2.
  • VCEP-pilot-variants---MMR.xlsx, full-text conversion: no matching row for c.232G>C, p.Glu78Gln, or p.E78Q.
Still missing
  • Required individual-level in-trans co-occurrence with a known pathogenic MLH1 variant, confirmed phase, qualifying age, and absence of CMMRD manifestations are unavailable.
  • VCEP-pilot-variants---MMR.xlsx was searched for c.232G>C, p.Glu78Gln, and p.E78Q; no entry for this variant was found.
Sources
BS3 VCEPNot assessed: no variant-specific proficient-function result or calibrated functional odds is available to compare with the VCEP BS3 thresholds. Details ▾Hide ▴
Reasoning

BS3 cannot be assessed because the case materials contain no variant-specific proficient-function result, calibrated functional odds, protein or mRNA assay result, or synonymous/intronic RNA result for MLH1 c.232G>C (p.Glu78Gln/p.E78Q). The governing VCEP rule permits BS3 Strong for calibrated functional odds <=0.05 or qualifying synonymous/intronic RNA evidence, and BS3 Supporting for calibrated functional odds >0.05 and <=0.48 or variant-specific proficient function in protein and mRNA-based assays following the MMR functional assay flowchart; none can be evaluated from the available evidence. The required VCEP assay documentation and flowchart were searched for c.232G>C, p.Glu78Gln, and p.E78Q, but neither contains a variant-specific entry. The only fetched paper, PMID:25394175, does not mention the variant and provides no functional assay evidence.

VCEP rule
Calibrated functional assays with functional odds for Pathogenicity <= 0.05 ... OR Synonymous substitutions and intronic variants with no associated mRNA aberration (either splicing or allelic imbalance) as determined by laboratory assays conducted with nonsense-mediated decay inhibition. Whenever abnormal transcripts are identified at similar levels in controls they will be considered naturally occurring isoforms and not mRNA aberrations. ... Calibrated functional assays with functional odds for Pathogenicity >0.05 & <=0.48 ... OR Variant-specific proficient function in protein and mRNA-based lab assays as per MMR functional assay flowchart*.
Evidence considered
  • CSPEC BS3 entries: calibrated functional odds <=0.05 or qualifying synonymous/intronic RNA evidence support Strong; calibrated functional odds >0.05 and <=0.48 or variant-specific proficient function in protein and mRNA-based assays following the MMR functional assay flowchart support Supporting.
  • Functional-assay-SVI-documentation-MMR.xlsx, sheet MMR assays: validated MLH1 assay rows and their control/threshold framework are listed, but no row identifies c.232G>C, p.Glu78Gln, or p.E78Q and no variant readout is provided.
  • Functional-assay-flowchart.pdf: the proficient-function pathway requires qualifying variant-specific assay evidence, which is not available for this case.
  • Searches of the two governing VCEP files for c.232G>C, p.Glu78Gln, and p.E78Q returned no variant-specific entry.
  • PMID:25394175 paper_extracts entry: the paper does not mention MLH1 c.232G>C or p.(E78Q); its quoted content concerns constitutional mismatch repair deficiency generally, not this variant or a functional assay.
Still missing
  • No variant-specific calibrated functional assay result or functional odds for MLH1 c.232G>C are available.
  • No variant-specific protein- or mRNA-based proficient-function result is available.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in Functional-assay-SVI-documentation-MMR.xlsx, sheet MMR assays.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in Functional-assay-flowchart.pdf.
  • PMID:25394175 was reviewed; its paper extract states that the variant is not mentioned and supplies no variant-specific functional evidence.
Sources
BS4 VCEPNot assessed: no non-segregating relatives, informative pedigree data, or combined Bayes Likelihood Ratio is available. Details ▾Hide ▴
Reasoning

The VCEP requires lack of co-segregation evaluated by a combined Bayes Likelihood Ratio, with strong evidence below 0.05 and supporting evidence above 0.05 through 0.48. The available case evidence contains no non-segregating pedigree observations, informative relative genotypes and phenotypes, or combined Bayes Likelihood Ratio for NM_000249.4:c.232G>C (p.Glu78Gln). BS4 therefore cannot be assessed.

VCEP rule
Lack of co-segregation with disease in pedigree(s) with a combined* Bayes Likelihood Ratio <0.05; Lack of co-segregation with disease in pedigree(s) with a combined* Bayes Likelihood Ratio >0.05 & ≤0.48
Evidence considered
  • No non-segregation observations, informative relative genotype/phenotype data, or combined Bayes Likelihood Ratio is documented in the available case evidence for NM_000249.4:c.232G>C (p.Glu78Gln).
Still missing
  • Non-segregating affected or unaffected relative observations
  • Relative genotypes and phenotype ascertainment
  • Combined Bayes Likelihood Ratio for lack of co-segregation
Sources
BP5 VCEPNot assessed: no tumor MSS, MMR-protein, BRAF V600E, or MLH1-methylation findings are available to establish an alternate molecular basis. Details ▾Hide ▴
Reasoning

BP5 is applicable, but the case contains no tumor-level or patient-level evidence needed to establish an alternate molecular basis. The VCEP requires either 2 or 3 qualifying tumors for BP5 Supporting—CRC/endometrial tumors with MSS and/or no MMR-protein loss, or LS-spectrum tumors with MMR-protein loss inconsistent with MLH1 variation—or one qualifying BRAF V600E or MLH1-methylated tumor pattern. No such findings are available. The Table-for-CMMRD-diagnosis.pdf and VCEP-pilot-variants---MMR.xlsx.txt were searched for c.232G>C, p.Glu78Gln, and p.E78Q; neither contains a variant-specific entry.

VCEP rule
2 or 3 tumors: CRC/Endometrial tumors with MSS and/or no loss of MMR protein expression and/or LS spectrum tumors<sup>d</sup> with loss of MMR protein(s) that is inconsistent with the gene demonstrating genetic variation. OR  1 BRAF V600E (Colon only)/_MLH1_ methylation (in LS spectrum tumor only) with MSI-H/_MLH1_ loss.
Evidence considered
  • The VCEP BP5 Supporting rule requires 2 or 3 qualifying tumors with MSS and/or no MMR-protein loss, or LS-spectrum tumors with MMR-protein loss inconsistent with the gene demonstrating variation; alternatively it requires 1 BRAF V600E or MLH1-methylated tumor pattern.
  • No qualifying tumor phenotype, MSS/MSI result, MMR-protein result, BRAF V600E result, MLH1-methylation result, or tumor count is present in the case evidence.
  • Table-for-CMMRD-diagnosis.pdf and VCEP-pilot-variants---MMR.xlsx were searched for all three variant notations and contained no variant-specific entry.
Still missing
  • Tumor-level evidence is missing: MSS/MSI status, MMR-protein expression, BRAF V600E status, MLH1 promoter methylation, tumor count, and tumor relationship to MLH1 variation.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in Table-for-CMMRD-diagnosis.pdf.txt.
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in VCEP-pilot-variants---MMR.xlsx.txt.
Sources
Not applicable · 12· recorded as not applicable Show reasons ▾Hide reasons ▴
PVS1 N/ANot applicable: c.232G>C is missense p.Glu78Gln, while the VCEP PVS1 rule is explicitly marked Not Applicable for this criterion. Details ▾Hide ▴
Reasoning

PVS1 is not applicable. NM_000249.4:c.232G>C is a missense variant, p.(Glu78Gln) / p.(E78Q), rather than a nonsense, frameshift, canonical splice, large genomic alteration, initiation-codon, or qualifying experimentally demonstrated splicing variant named by the MLH1 PVS1 rule. The VCEP's PVS1 criterion is explicitly marked Not Applicable for this case. No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in PVS1_DecisionTree_MMR.pdf.txt or VCEP-pilot-variants---MMR.xlsx.txt.

VCEP rule
Not Applicable
Evidence considered
  • VCEP CSPEC v2.0 criterion entry for PVS1: applicability is "Not Applicable".
  • Case normalization identifies NM_000249.4:c.232G>C as NP_000240.1:p.(Glu78Gln) / p.(E78Q), consequence class missense.
  • The required searches found no matching c.232G>C, p.Glu78Gln, or p.E78Q row in PVS1_DecisionTree_MMR.pdf.txt or VCEP-pilot-variants---MMR.xlsx.txt.
Still missing
  • No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in /Users/gs_agent/LYFESCI_HERMES_v11/vcep_db/MLH1/fulltext/PVS1_DecisionTree_MMR.pdf.txt.
  • No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in /Users/gs_agent/LYFESCI_HERMES_v11/vcep_db/MLH1/fulltext/VCEP-pilot-variants---MMR.xlsx.txt.
Sources
PS4 VCEPNot applicable: the MLH1 VCEP excludes proband-counting PS4 because tumor IHC evidence is evaluated through PP4. Details ▾Hide ▴
Reasoning

The MLH1 VCEP marks PS4 not applicable because tumor IHC data are available for variant classification through PP4; it therefore does not use proband-counting PS4 for MMR variant classification. The case literature packet contains no AI-classified PS4 papers. PMID:25394175 was reviewed because it was indexed as a PS4 lead, but it is a cancer-genetics referral guideline, does not mention NM_000249.4:c.232G>C, p.Glu78Gln, or p.E78Q, and provides no variant-specific case-control counts.

VCEP rule
Due to the availability of tumor IHC data for variant classification (see PP4), PS4 has not been utilized for MMR variant classification using proband counting.
Evidence considered
  • The governing MLH1 VCEP states that PS4 is not applicable because tumor IHC data are available for classification through PP4.
  • PMID:25394175 was indexed as a PS4 lead and was read in full; it is a cancer-genetics referral guideline and does not mention c.232G>C, p.Glu78Gln, or p.E78Q or provide variant-specific case-control data.
Still missing
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in VCEP-pilot-variants---MMR.xlsx.txt; this does not override the VCEP PS4 applicability rule.
Sources
PM1 VCEPNot applicable: the MLH1 VCEP explicitly marks PM1 Not Applicable despite residue 78 lying within listed functional domains. Details ▾Hide ▴
Reasoning

The governing MLH1 VCEP marks PM1 Not Applicable. Although residue 78 falls within the MLH1 ATPase domain (residues 20-147) and MutS-alpha interaction domain (residues 1-329) in the declared MMR-Functional-domains.pdf schematic, the VCEP's explicit applicability designation controls. vcep_materials.json has no domain_tables entry for MLH1, and the searched domain file contains no exact c.232G>C, p.Glu78Gln, or p.E78Q row.

VCEP rule
Not Applicable
Evidence considered
  • The MLH1 VCEP CSPEC entry for PM1 is explicitly marked Not Applicable.
  • MMR-Functional-domains.pdf.txt lists MLH1 ATPase residues 20-147 and MutS-alpha interaction residues 1-329; residue 78 is within both ranges.
  • Searches of MMR-Functional-domains.pdf.txt for c.232G>C, p.Glu78Gln, and p.E78Q found no exact variant entry.
  • vcep_materials.json has domain_tables={}; therefore no MLH1 domain_tables entry supersedes the VCEP PM1 applicability designation.
Sources
PM4 VCEPNot applicable: the VCEP explicitly marks PM4 Not Applicable, and c.232G>C is a missense substitution without a VCEP PM4 path. Details ▾Hide ▴
Reasoning

PM4 is not applicable because the VCEP's MLH1 CSPEC criterion entry explicitly marks PM4 as Not Applicable. The variant is a missense substitution, p.(Glu78Gln), not a protein-length change assessment path under this VCEP specification. No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in PVS1_DecisionTree_MMR.pdf.txt or VCEP-pilot-variants---MMR.xlsx.txt.

VCEP rule
Not Applicable
Evidence considered
  • VCEP CSPEC v2.0 criterion entry for PM4: applicability is "Not Applicable".
  • Case normalization identifies NM_000249.4:c.232G>C as NP_000240.1:p.(Glu78Gln) / p.(E78Q), consequence class missense.
  • The required searches found no matching c.232G>C, p.Glu78Gln, or p.E78Q row in PVS1_DecisionTree_MMR.pdf.txt or VCEP-pilot-variants---MMR.xlsx.txt.
Still missing
  • No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in /Users/gs_agent/LYFESCI_HERMES_v11/vcep_db/MLH1/fulltext/PVS1_DecisionTree_MMR.pdf.txt.
  • No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in /Users/gs_agent/LYFESCI_HERMES_v11/vcep_db/MLH1/fulltext/VCEP-pilot-variants---MMR.xlsx.txt.
Sources
PM6 VCEPNot applicable: the MLH1 VCEP explicitly marks PM6 as not applicable. Details ▾Hide ▴
Reasoning

The ClinGen InSiGHT MLH1 VCEP specification explicitly marks PM6 as Not Applicable. No PM6 evidence is considered under this governing framework.

VCEP rule
Not Applicable
Evidence considered
  • ClinGen InSiGHT MLH1 VCEP CSPEC criterion entry for PM6: applicability is listed as Not Applicable.
Sources
PP2 VCEPNot applicable: the MLH1 VCEP explicitly marks PP2 Not Applicable. Details ▾Hide ▴
Reasoning

The governing MLH1 VCEP explicitly marks PP2 Not Applicable. No generic gene-level missense mechanism rule was substituted.

VCEP rule
Not Applicable
Evidence considered
  • The MLH1 VCEP CSPEC entry for PP2 is explicitly marked Not Applicable.
Sources
PP5 VCEPNot applicable: the MLH1 VCEP prohibits PP5, regardless of the single-submitter ClinVar classification or referral-guideline citation. Details ▾Hide ▴
Reasoning

The MLH1 VCEP marks PP5 not applicable for this VCEP and states that the criterion is not for use as recommended by the ClinGen Sequence Variant Interpretation VCEP Review Committee. The ClinVar record is a single-submitter uncertain-significance submission, not usable expert-panel evidence. PMID:25394175 was reviewed because it was indexed as a PP5 lead; it is a referral guideline, does not mention this variant, and cannot establish PP5.

VCEP rule
This criterion is not for use as recommended by the ClinGen Sequence Variant Interpretation VCEP Review Committee.
Evidence considered
  • The governing MLH1 VCEP states that PP5 is not applicable for this VCEP and is not for use as recommended by the ClinGen Sequence Variant Interpretation VCEP Review Committee.
  • ClinVar contains one single-submitter uncertain-significance submission for variation 1789704 and no expert-panel submission.
  • PMID:25394175 was read in full and does not mention c.232G>C, p.Glu78Gln, or p.E78Q.
Still missing
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in VCEP-pilot-variants---MMR.xlsx.txt; this does not override the VCEP PP5 applicability rule.
Sources
BP1 VCEPNot applicable: the MLH1 VCEP explicitly marks BP1 Not Applicable. Details ▾Hide ▴
Reasoning

The governing MLH1 VCEP explicitly marks BP1 Not Applicable. No generic benign missense-in-gene rule was substituted.

VCEP rule
Not Applicable
Evidence considered
  • The MLH1 VCEP CSPEC entry for BP1 is explicitly marked Not Applicable.
Sources
BP2 VCEPNot applicable: the MLH1 VCEP marks BP2 not applicable and directs that BS2 is used instead. Details ▾Hide ▴
Reasoning

The ClinGen InSiGHT MLH1 VCEP marks BP2 as not applicable and directs that BS2 is used instead. BP2 is therefore not evaluated, regardless of any available phase or co-occurrence information.

VCEP rule
BS2 is used instead.
Evidence considered
  • The VCEP criterion entry lists BP2 as not applicable and states: “BS2 is used instead.”
Sources
BP3 VCEPNot applicable: the VCEP explicitly marks BP3 Not Applicable, and c.232G>C is missense rather than an applicable repeat-region in-frame indel. Details ▾Hide ▴
Reasoning

BP3 is not applicable because the VCEP's MLH1 CSPEC criterion entry explicitly marks BP3 as Not Applicable. The variant is a missense substitution, p.(Glu78Gln), rather than a benign in-frame deletion or insertion in a repetitive region under an applicable VCEP rule. No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in PVS1_DecisionTree_MMR.pdf.txt or VCEP-pilot-variants---MMR.xlsx.txt.

VCEP rule
Not Applicable
Evidence considered
  • VCEP CSPEC v2.0 criterion entry for BP3: applicability is "Not Applicable".
  • Case normalization identifies NM_000249.4:c.232G>C as NP_000240.1:p.(Glu78Gln) / p.(E78Q), consequence class missense.
  • The required searches found no matching c.232G>C, p.Glu78Gln, or p.E78Q row in PVS1_DecisionTree_MMR.pdf.txt or VCEP-pilot-variants---MMR.xlsx.txt.
Still missing
  • No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in /Users/gs_agent/LYFESCI_HERMES_v11/vcep_db/MLH1/fulltext/PVS1_DecisionTree_MMR.pdf.txt.
  • No matching c.232G>C, p.Glu78Gln, or p.E78Q entry was found in /Users/gs_agent/LYFESCI_HERMES_v11/vcep_db/MLH1/fulltext/VCEP-pilot-variants---MMR.xlsx.txt.
Sources
BP6 VCEPNot applicable: the MLH1 VCEP prohibits BP6, and the available ClinVar submission is uncertain significance rather than a qualifying benign report. Details ▾Hide ▴
Reasoning

The MLH1 VCEP marks BP6 not applicable and states that this criterion is not for use as recommended by the ClinGen Sequence Variant Interpretation VCEP Review Committee. The available ClinVar record is uncertain significance from one clinical laboratory and supplies no reputable benign assertion that could change the VCEP applicability decision.

VCEP rule
This criterion is not for use as recommended by the ClinGen Sequence Variant Interpretation VCEP Review Committee.
Evidence considered
  • The governing MLH1 VCEP states that BP6 is not applicable for this VCEP and is not for use as recommended by the ClinGen Sequence Variant Interpretation VCEP Review Committee.
  • ClinVar contains one single-submitter uncertain-significance submission for variation 1789704, with no expert-panel submission or benign classification.
Still missing
  • No entry for c.232G>C, p.Glu78Gln, or p.E78Q was found in VCEP-pilot-variants---MMR.xlsx.txt; this does not override the VCEP BP6 applicability rule.
Sources
BP7 N/ANot applicable: c.232G>C causes the missense substitution p.Glu78Gln, whereas BP7 covers only synonymous or intronic variants. Details ▾Hide ▴
Reasoning

The variant is a missense substitution, c.232G>C (p.Glu78Gln), rather than a synonymous or intronic variant. BP7 is therefore outside its VCEP-defined scope and is not applicable; no predictor score is needed.

VCEP rule
A synonymous (silent) or intronic variant at or beyond -21/+7 (5′/3′ exonic). Variants may satisfy both BP7 and BP4.
Evidence considered
  • Case normalization identifies NM_000249.4:c.232G>C as NP_000240.1:p.(Glu78Gln), a missense variant.
Sources

Identifiers

Gene
MLH1
Transcript
NM_000249.4
Coding (HGVS c.)
NM_000249.4:c.232G>C
Protein (HGVS p.)
NP_000240.1:p.(Glu78Gln)
GRCh38
chr3:37000979 G>C
GRCh37
chr3:37042470 G>C
Location and type
Exon 3 · missense
ClinVar
1789704

Gene diagram

Every interpreted variant in MLH1, mapped onto the exon structure of NM_000249.4. Select a row to find it on the plot.

MLH1NM_000249.4
Fetching transcript structure from UCSC…
gnomAD v4.1 2 / 1,607,590 alleles AF 1.24e-06 · 0 homozygotes · FAF 3.65e-06
gnomAD v2.1 Allele count data not recorded
gnomAD-Canada v1.0 Allele count data not recorded

By genetic ancestry

gnomAD v4.1

AF 1.2e-06 · 2 / 1,607,590 alleles · 0 hom · FAF 3.7e-06
South Asian2 / 90,930 2.2e-05
0 alternate alleles recorded in 9 other groups: Remaining individuals, Admixed American, European (Finnish), Amish, East Asian, Middle Eastern, Ashkenazi Jewish, African/African American, European (non-Finnish).

Statements

gnomAD v4.1

This variant is present in gnomAD v4.1 (AF= 1.2441e-06; MAF= 0.00012%, 2/1607590 alleles, homozygotes = 0) and has highest observed frequency in the South Asian population (AF= 2.19949e-05; MAF= 0.00220%, 2/90930 alleles, homozygotes = 0); grpmax FAF= 3.65e-06.

gnomAD v2.1

Absent from gnomAD v2.1.

gnomAD-Canada

Not available in gnomAD-Canada v1.0.

SpliceAI and Pangolin

SpliceAI acceptor lossat -24 bp
0.009
SpliceAI acceptor gainat +5 bp
0.000
SpliceAI donor lossat +69 bp
0.040
SpliceAI donor gainat +4 bp
0.000
Pangolin splice lossmagnitude shown
-0.012
Pangolin splice gain
0.003

Other predictors

REVEL
0.855
BayesDel
0.432

How the predictions were used

PP3 SupportingVCEPMet at Supporting: HCI prior probability is 0.7144, within the VCEP PP3 Supporting range (>0.68 and ≤0.81). Details ▾Hide ▴
Reasoning

The variant is a missense substitution, so the VCEP's missense HCI-prior pathway applies. The governing HCI-PRIORS-MLH1 table contains the exact entry c.232G>C / p.E78Q with prior probability 0.7144. This is greater than 0.68 and no greater than 0.81, satisfying PP3 at Supporting strength. The table's pre-assigned result governs; REVEL, BayesDel, and the SpliceAI no-impact score are not used for this PP3 decision.

VCEP rule
Missense variant with HCI prior probability for pathogenicity >0.68 & ≤0.81 as per [https://hci-priors.hci.utah.edu/PRIORS](https://hci-priors.hci.utah.edu/PRIORS) OR Predicted splice defect for non-canonical splicing nucleotides using SpliceAI with delta score >= 0.2 as per Walker et al 2023.
Evidence considered
  • HCI-PRIORS-MLH1.txt.txt, exact row for c.232G>C / p.E78Q: prior probability of pathogenicity = 0.7144, MAPP score = 13.2, PMID22949387 listed in the row.
  • The VCEP CSPEC identifies missense HCI prior probability >0.68 and ≤0.81 as PP3 Supporting; the exact table entry is therefore applied directly.
Sources
BP4 VCEPNot met: HCI prior probability is 0.7144, not below the VCEP BP4 cutoff of 0.11 for missense variants. Details ▾Hide ▴
Reasoning

The variant is missense, so the VCEP's HCI-prior pathway is the applicable BP4 path. The exact HCI-PRIORS-MLH1 entry for c.232G>C / p.E78Q gives a pathogenicity prior of 0.7144, not less than 0.11; therefore BP4 Supporting is not met. The VCEP's alternative SpliceAI no-impact path is explicitly limited to intronic and synonymous variants and is not applicable to this missense variant.

VCEP rule
Missense variant with HCI-prior probability of pathogenicity \<0.11 as per [https://hci-priors.hci.utah.edu/PRIORS](https://hci-priors.hci.utah.edu/PRIORS) OR For intronic and synonymous variants: SpliceAI predicts no splicing impact with delta score \<= 0.1 as per Walker et al 2023
Evidence considered
  • HCI-PRIORS-MLH1.txt.txt, exact row for c.232G>C / p.E78Q: prior probability of pathogenicity = 0.7144.
  • SpliceAI max delta score = 0.04 is present in the case evidence, but the VCEP BP4 SpliceAI alternative applies only to intronic and synonymous variants, not this missense substitution.
Sources
BP7 N/ANot applicable: c.232G>C causes the missense substitution p.Glu78Gln, whereas BP7 covers only synonymous or intronic variants. Details ▾Hide ▴
Reasoning

The variant is a missense substitution, c.232G>C (p.Glu78Gln), rather than a synonymous or intronic variant. BP7 is therefore outside its VCEP-defined scope and is not applicable; no predictor score is needed.

VCEP rule
A synonymous (silent) or intronic variant at or beyond -21/+7 (5′/3′ exonic). Variants may satisfy both BP7 and BP4.
Evidence considered
  • Case normalization identifies NM_000249.4:c.232G>C as NP_000240.1:p.(Glu78Gln), a missense variant.
Sources

Statement

In silico

SpliceAI predicts no significant splice impact for this variant (max delta score = 0.04). REVEL score = 0.855. BayesDel score = 0.43245. HCI prior probability for pathogenicity = 0.7144. MAPP score = 13.2. Custom PP2 score = 1.0.

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.

Recorded publications

  • A practice guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: referral indications for cancer predisposition assessment.
    Hampel H et al. · 2015
    The source does not mention MLH1 c.232G>C or p.(E78Q); it discusses constitutional mismatch repair deficiency generally as a recessive condition caused by biallelic mismatch-repair-gene mutations, including MLH1, and does not provide variant-specific functional evidence.
    Does not name this variant
    PMID 25394175 ↗
    Quoted passage
    Constitutional mismatch repair deficiency is a recessive condition caused by biallelic mutations in the mismatch repair genes (MLH1, MSH2 (including methylation due to an EPCAM deletion), MSH6, and PMS2) and is characterized by a high risk of developing cancers during childhood, including Lynch syndrome (LS)–associated cancers, hematologic malignancies, and embryonic tumors.

    Recommendations, section "Constitutional mismatch repair deficiency (OMIM 276300)", page 77

Screenshots represent source records captured during evidence collection. Select one to view the recorded source content.

All source links

Statements for reports

The evidence statements recorded by the interpretation pipeline. They have not been reinterpreted by the page.

gnomAD v4.1

This variant is present in gnomAD v4.1 (AF= 1.2441e-06; MAF= 0.00012%, 2/1607590 alleles, homozygotes = 0) and has highest observed frequency in the South Asian population (AF= 2.19949e-05; MAF= 0.00220%, 2/90930 alleles, homozygotes = 0); grpmax FAF= 3.65e-06.

gnomAD v2.1

Absent from gnomAD v2.1.

gnomAD-Canada

Not available in gnomAD-Canada v1.0.

ClinVar

This variant has been reported in ClinVar as Uncertain significance (1 clinical laboratory). (ClinVarID = 1789704)

In silico

SpliceAI predicts no significant splice impact for this variant (max delta score = 0.04). REVEL score = 0.855. BayesDel score = 0.43245. HCI prior probability for pathogenicity = 0.7144. MAPP score = 13.2. Custom PP2 score = 1.0.

Functional

OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. MLH1, a DNA mismatch repair protein, is recurrently altered by deletion and mutation in various cancer types.

Somatic

This variant does not lie in a statistically significant hotspot. This variant has not previously been reported in somatic cancers (COSMIC).

Cancer Hotspots

This variant does not lie in a statistically significant hotspot.