Genetic Information

Gene & Transcript Details

Gene
KRAS
Transcript
NM_001369786.1 MANE Select
Total Exons
Reference Sequence
NC_000012.11
Alternative Transcripts
IDStatusDetails
NM_001369786.1 Alternative 5417 nt | 178–747

Variant Details

HGVS Notation
NM_001369786.1:c.194G>T
Protein Change
S65I
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
Likely Pathogenic
2 publications
Publications List
PMID: 25326635

This sequence change replaces serine, which is neutral and polar, with isoleucine, which is neutral and non-polar, at codon 65 of the KRAS protein (p.Ser65Ile). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with clinical features of KRAS-related conditions (PMID: 25326635, 26822237). In at least one individual the variant was observed to be de novo. ClinVar contains an entry for this variant (Variation ID: 438796). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt KRAS protein function. For these reasons, this variant has been classified as Pathogenic.

Clinical Statement

"This variant has been reported in ClinVar as Uncertain significance (1 clinical laboratories) and as Pathogenic (1 clinical laboratories) and as Likely pathogenic by ClinGen RASopathy Variant Curation Expert Panel expert panel."

COSMIC Somatic Evidence

Open
COSMIC ID
COSM5044326
Recurrence
2 occurrences
PM1 Criteria
Not 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

Error in OpenAI Consolidation. OncoKB: KRASS65IKRASS65ISomaticNCBI Gene:3845|Show additional gene information Variant OverviewKRAS, a GTPase which functions as an upstream regulator of the MAPK pathway, is frequently mutated in various cancer types including lung, colorectal and pancreatic cancers.The KRAS S65I mutation has not specifically been reviewed by the OncoKB team. However, KRAS S65N is known to be oncogenic, and therefore KRAS S65I is considered likely oncogenic.Hide mutation effect description The KRAS S65I mutation has not specifically been reviewed by the OncoKB team. However, the mutation effect description for KRAS S65N, an alternate allele of KRAS S65I, is: The KRAS S65N mutation is located in the Switch II region of the catalytic G-domain of the protein. This mutation has been found in thyroid cancer (PMID: 24798740). Expression of this mutation in human embryonic kidney and thyroid cancer cells demonstrated that it is activating, as measured by increased pathway activation and cell proliferation compared to wildtype (PMID: 8626650, 24798740). JAX-CKB: No results found

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 None bp
- Donor Loss (DL) 0.0 None bp
+ Acceptor Gain (AG) 0.0 None bp
+ Donor Gain (DG) 0.0 None 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:
PS3

PS3 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)

PM2

PM2 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)

PP5

PP5 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)