Genetic Information

Gene & Transcript Details

Gene
TERT
Transcript
NM_198253.2 MANE Select
Total Exons
Reference Sequence
NC_000005.9
Alternative Transcripts
IDStatusDetails
NM_198253.3 MANE Select 4039 nt | 80–3478
NM_198253.1 Alternative 3644 nt | 56–2479
NM_198253.2 RefSeq Select 4018 nt | 59–3457

Variant Details

HGVS Notation
NM_198253.2:c.835G>A
Protein Change
A279T
Location
Exon 2 (Exon 2 of )
2
5'Exon Structure3'
Functional Consequence
Loss of Function
Alternate Identifiers

Clinical & Population Data

Population Frequency

gnomAD
Global Frequency
2.31 in 100,000
Extremely Rare
ACMG Criteria Applied BA1

ClinVar

Open
Classification
Benign
1 publications
Publications List
Clinical Statement

"This variant has been reported in ClinVar as Benign (18 clinical laboratories)."

COSMIC Somatic Evidence

Open
COSMIC ID
COSM3760906
Recurrence
11 occurrences
PM1 Criteria
Not Applied
COSMIC Database 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 TERT.

Functional Studies & Therapeutic Relevance

Functional Summary

Error in OpenAI Consolidation. OncoKB: TERTA279TTERTA279TSomaticNCBI Gene:7015|Show additional gene information Variant OverviewTERT is an enzyme that functions to maintain telomere length and genomic stability. The TERT promoter is frequently mutated in various cancer types.The TERT A279T mutation has not specifically been reviewed by the OncoKB team, and therefore its biological significance is unknown. 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 -172 bp
- Donor Loss (DL) 0.0 328 bp
+ Acceptor Gain (AG) 0.0 -7 bp
+ Donor Gain (DG) 0.0 -24 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:
BP4

BP4 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)

BP6

BP6 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)