Genetic Information

Gene & Transcript Details

Gene
KRAS
Transcript
NM_033360.2 MANE Select
Total Exons
Reference Sequence
NC_000012.11
Alternative Transcripts
IDStatusDetails
NM_033360.2 Alternative 5436 nt | 182–751
NM_033360.4 Alternative 5430 nt | 191–760
NM_033360.3 Alternative 5889 nt | 193–762

Variant Details

HGVS Notation
NM_033360.2:c.175G>A
Protein Change
A59T
Location
Exon 3 (Exon 3 of )
3
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
Not Classified
0 publications
Clinical Statement

"Present in ClinVar, however no clinical evidence available for this variant."

COSMIC Somatic Evidence

Open
COSMIC ID
COSM546
Recurrence
47 occurrences
PM1 Criteria
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 KRAS.

Functional Studies & Therapeutic Relevance

Functional Summary

The KRAS A59T variant has been functionally characterized as a gain-of-function mutation. Experimental evidence demonstrates that this variant is oncogenic, leading to increased cell proliferation and migration in NIH3T3 cells, as well as enhanced MAPK signaling in Ba/F3 cells compared to the wildtype. These findings support a damaging effect of the KRAS A59T mutation.

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
Yes
REVEL Score
0.869
Threshold: ≥0.75 = PP3 applied

SpliceAI Scores

Window: ±500bp
Effect Type Score Position
- Acceptor Loss (AL) 0.0 -1 bp
- Donor Loss (DL) 0.0 -65 bp
+ Acceptor Gain (AG) 0.01 -4 bp
+ Donor Gain (DG) 0.07 -3 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 VCEP guidelines, PVS1 addresses null variants in genes where loss-of-function is a known mechanism. KRAS-associated disease mechanism is gain-of-function and this variant is a missense change. The evidence shows a missense alteration (A59T). Therefore, this criterion is not applied.

PS1

PS1 (Not Applied)

According to VCEP guidelines, PS1 (Strong) requires the same amino acid change as a previously established pathogenic variant. The evidence shows no identical A59T nucleotide change previously reported as pathogenic. Therefore, this criterion is not applied.

PS2

PS2 (Not Applied)

According to standard ACMG guidelines, PS2 (Strong) applies to de novo occurrences with confirmed parentage. No de novo data are available for this variant. Therefore, this criterion is not applied.

PS3

PS3 (Moderate)

According to VCEP guidelines, PS3 (Moderate) is met by two or more different approved functional assays. The evidence shows oncogenic gain-of-function demonstrated by increased cell proliferation in NIH3T3 cells and enhanced MAPK signaling in Ba/F3 cells. Therefore, this criterion is applied at Moderate strength.

PS4

PS4 (Not Applied)

According to VCEP guidelines, PS4 (Strong) requires ≥5 proband points. No case-level data or point counts are available. Therefore, this criterion is not applied.

PM1

PM1 (Moderate)

According to VCEP guidelines, PM1 (Moderate) applies to variants in the SW2 region (AA 57–64). A59T lies within SW2. Therefore, this criterion is applied at Moderate strength.

PM2

PM2 (Supporting)

According to VCEP guidelines, PM2 (Supporting) applies when the variant is absent from gnomAD. The evidence shows A59T is not present in gnomAD. Therefore, this criterion is applied at Supporting strength.

PM3

PM3 (Not Applied)

According to standard ACMG guidelines, PM3 (Moderate) applies to recessive disorders with detected trans variants. KRAS A59T is associated with a dominant oncogenic mechanism, and no trans data are available. Therefore, this criterion is not applied.

PM4

PM4 (Not Applied)

According to standard ACMG guidelines, PM4 (Moderate) applies to in-frame insertions/deletions or stop-loss variants. A59T is a missense change. Therefore, this criterion is not applied.

PM5

PM5 (Moderate)

According to VCEP guidelines, PM5 (Moderate) applies when one other pathogenic residue change at the same codon is reported. A59G has been established as pathogenic. Therefore, this criterion is applied at Moderate strength.

PM6

PM6 (Not Applied)

According to standard ACMG guidelines, PM6 (Supporting) applies to assumed de novo variants without confirmation. No de novo evidence is available. Therefore, this criterion is not applied.

PP1

PP1 (Not Applied)

According to standard ACMG guidelines, PP1 (Supporting) requires segregation data. No family segregation data are available. Therefore, this criterion is not applied.

PP2

PP2 (Not Applied)

According to standard ACMG guidelines, PP2 (Supporting) applies when a gene has a low rate of benign missense variation. No specific constraint metrics or gene-level data were provided. Therefore, this criterion is not applied.

PP3

PP3 (Supporting)

According to VCEP guidelines, PP3 (Supporting) applies for missense variants with REVEL ≥0.7. The evidence shows a REVEL score of 0.87. Therefore, this criterion is applied at Supporting strength.

PP4

PP4 (Not Applied)

According to standard ACMG guidelines, PP4 (Supporting) requires a highly specific phenotype. No phenotype data are provided. Therefore, this criterion is not applied.

PP5

PP5 (Not Applied)

According to standard ACMG guidelines, PP5 (Supporting) applies when reputable sources report the variant as pathogenic without evidence. ClinVar has no assertion for this variant. Therefore, this criterion is not applied.

BA1

BA1 (Not Applied)

According to standard ACMG guidelines, BA1 (Stand Alone) requires population allele frequency ≥5%. The variant is absent from controls. Therefore, this criterion is not applied.

BS1

BS1 (Not Applied)

According to standard ACMG guidelines, BS1 (Strong) requires allele frequency ≥2.5%. The variant is absent from controls. Therefore, this criterion is not applied.

BS2

BS2 (Not Applied)

According to standard ACMG guidelines, BS2 (Strong/Supporting) applies when observed in multiple healthy individuals. No such data are available. Therefore, this criterion is not applied.

BS3

BS3 (Not Applied)

According to standard ACMG guidelines, BS3 (Strong) applies when well-established functional studies show no damaging effect. Functional studies for A59T demonstrate gain-of-function. Therefore, this criterion is not applied.

BS4

BS4 (Not Applied)

According to standard ACMG guidelines, BS4 (Strong) requires non-segregation in affected members. No segregation data are available. Therefore, this criterion is not applied.

BP1

BP1 (Not Applied)

According to standard ACMG guidelines, BP1 (Supporting) applies to truncating variants in genes not associated with loss-of-function. A59T is missense. Therefore, this criterion is not applied.

BP2

BP2 (Not Applied)

According to standard ACMG guidelines, BP2 (Supporting) applies to observation in trans with a pathogenic variant in dominant disorders. No such data are available. Therefore, this criterion is not applied.

BP3

BP3 (Not Applied)

According to standard ACMG guidelines, BP3 (Supporting) applies to in-frame indels in repetitive regions. A59T is a missense change. Therefore, this criterion is not applied.

BP4

BP4 (Not Applied)

According to VCEP guidelines, BP4 (Supporting) applies for missense variants with REVEL ≤0.3. The REVEL score is 0.87, well above threshold. Therefore, this criterion is not applied.

BP5

BP5 (Not Applied)

According to standard ACMG guidelines, BP5 (Supporting) applies when a variant is found in a case with an alternate molecular cause. No alternate cause data are available. Therefore, this criterion is not applied.

BP6

BP6 (Not Applied)

According to standard ACMG guidelines, BP6 (Supporting) applies when reputable sources report as benign without evidence. No such reports exist. Therefore, this criterion is not applied.

BP7

BP7 (Not Applied)

According to standard ACMG guidelines, BP7 (Supporting) applies to synonymous changes with no predicted splice impact. A59T is missense. Therefore, this criterion is not applied.