Genetic Information

Gene & Transcript Details

Gene
BRAF
Transcript
NM_001354609.1 MANE Select
Total Exons
Reference Sequence
NC_000007.13
Alternative Transcripts
IDStatusDetails
NM_001354609.2 Alternative 9687 nt | 227–2530
NM_001354609.1 Alternative 9702 nt | 226–2529

Variant Details

HGVS Notation
NM_001354609.1:c.722C>A
Protein Change
T241K
Location
Exon 6 (Exon 6 of )
6
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: 17704260

In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. This variant disrupts the p.Thr241 amino acid residue in BRAF. Other variant(s) that disrupt this residue have been determined to be pathogenic (PMID: 17704260, 18042262, 19206169, 28404629). This suggests that this residue is clinically significant, and that variants that disrupt this residue are likely to be disease-causing. Advanced modeling performed at Invitae incorporating data from internal and/or published experimental studies (Invitae) indicates that this missense variant is expected to disrupt BRAF function. ClinVar contains an entry for this variant (Variation ID: 44829). This missense change has been observed in individual(s) with clinical features of RASopathy spectrum disorders (Invitae). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces threonine, which is neutral and polar, with lysine, which is basic and polar, at codon 241 of the BRAF protein (p.Thr241Lys).

Clinical Statement

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

COSMIC Somatic Evidence

Open
COSMIC ID
Recurrence
0 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 BRAF.

Functional Studies & Therapeutic Relevance

Functional Summary

Error in OpenAI Consolidation. OncoKB: BRAFT241KBRAFT241KSomaticNCBI Gene:673|Show additional gene information Variant OverviewBRAF, an intracellular kinase, is frequently mutated in melanoma, thyroid and lung cancers among others.The BRAF T241K mutation has not specifically been reviewed by the OncoKB team. However, BRAF T241P is likely oncogenic, and therefore BRAF T241K is considered likely oncogenic.Hide mutation effect description The BRAF T241K mutation has not specifically been reviewed by the OncoKB team. However, the mutation effect description for BRAF T241P, an alternate allele of BRAF T241K, is: The BRAF T241P mutation is located in the cysteine-rich domain in exon 6 of the protein. This mutation has been found in cardio-facio-cutaneous syndrome (CFC) (PMID: 19206169). Expression of this mutation in NIH-3T3 cells demonstrated that it is likely activating as measured by moderately increased activation of MEK and ERK and slightly increased colony formation compared to wildtype BRAF (PMID: 19206169). 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 -15 bp
- Donor Loss (DL) 0.0 -138 bp
+ Acceptor Gain (AG) 0.04 -38 bp
+ Donor Gain (DG) 0.0 -142 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)

BP4

BP4 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)