Genetic Information

Gene & Transcript Details

Gene
TP53
Transcript
NM_000546.6 MANE Select
Total Exons
Reference Sequence
NC_000017.10
Alternative Transcripts
IDStatusDetails
NM_000546.5 RefSeq Select 2591 nt | 203–1384
NM_000546.3 Alternative 2640 nt | 252–1433
NM_000546.6 MANE Select 2512 nt | 143–1324
NM_000546.4 Alternative 2586 nt | 198–1379
NM_000546.2 Alternative 2629 nt | 252–1433

Variant Details

HGVS Notation
NM_000546.6:c.-29+121G>T
Protein Change
?
Location
Exon 1 (Exon 1 of )
1
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
Unknown
0 publications
Clinical Statement

""

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 TP53.

Functional Studies & Therapeutic Relevance

Functional Summary

The TP53 -29+121G>T variant has not been functionally characterized.

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 -286 bp
- Donor Loss (DL) 0.0 202 bp
+ Acceptor Gain (AG) 0.0 -2 bp
+ Donor Gain (DG) 0.0 -2 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, 'Canonical splice variants (±1,2 intronic positions): PVS1 applies to predicted splicing alterations...' The variant c.-29+121G>T is located at +121 relative to the exon, outside the canonical ±1,2 splice sites. Therefore, PVS1 is not applied.

PS1

PS1 (Not Applied)

According to VCEP guidelines, PS1 applies when a variant results in the same amino acid change as a previously established pathogenic variant. This deep intronic variant has no protein change. Therefore, PS1 is not applied.

PS2

PS2 (Not Applied)

According to VCEP guidelines, PS2 requires a confirmed de novo occurrence (maternity and paternity confirmed). No de novo data are available for this variant. Therefore, PS2 is not applied.

PS3

PS3 (Not Applied)

According to VCEP guidelines, PS3 requires well-established functional studies showing loss of function in TP53-specific assays. No functional studies have been performed for this variant. Therefore, PS3 is not applied.

PS4

PS4 (Not Applied)

According to VCEP guidelines, PS4 requires case–control or proband count evidence with defined point thresholds. No case data are available. Therefore, PS4 is not applied.

PM1

PM1 (Not Applied)

According to VCEP guidelines, PM1 applies to missense variants in TP53 hotspots (codons 175,245,248,249,273,282) or recurrent somatic hotspots. This intronic variant does not affect a hotspot codon. Therefore, PM1 is not applied.

PM2

PM2 (Supporting)

According to VCEP guidelines, 'PM2 applies at Supporting strength for TP53 if allele frequency <0.00003 in gnomAD.' The variant is absent from gnomAD (MAF=0%). Therefore, PM2 is applied at Supporting strength.

PM3

PM3 (Not Applied)

According to standard ACMG guidelines, PM3 applies to detected in trans observations in a recessive disorder. TP53 is not recessive and no trans data exist. Therefore, PM3 is not applied.

PM4

PM4 (Not Applied)

According to standard ACMG guidelines, PM4 applies to protein length changes such as in-frame indels. This is an intronic variant not affecting coding sequence. Therefore, PM4 is not applied.

PM5

PM5 (Not Applied)

According to VCEP guidelines, PM5 applies to novel missense changes at residues with established pathogenic missense variants. This is noncoding. Therefore, PM5 is not applied.

PM6

PM6 (Not Applied)

According to VCEP guidelines, PM6 applies to presumed de novo without confirmation. No de novo evidence is available. Therefore, PM6 is not applied.

PP1

PP1 (Not Applied)

According to VCEP guidelines, PP1 requires segregation data across affected family members. No segregation data are available. Therefore, PP1 is not applied.

PP2

PP2 (Not Applied)

According to standard ACMG guidelines, PP2 applies to missense variants in genes with low benign missense variation rate. This is an intronic variant. Therefore, PP2 is not applied.

PP3

PP3 (Not Applied)

According to VCEP guidelines, PP3 applies to missense or splice region variants with in silico evidence of impact (SpliceAI ≥0.2). SpliceAI predicts no impact (score=0). Therefore, PP3 is not applied.

PP4

PP4 (Not Applied)

According to VCEP guidelines, PP4 relates to somatic VAF observations. This is a germline assessment with no somatic data. Therefore, PP4 is not applied.

PP5

PP5 (Not Applied)

According to standard ACMG guidelines, PP5 applies when a reputable source classifies a variant as pathogenic. No such classification exists for this variant. Therefore, PP5 is not applied.

BA1

BA1 (Not Applied)

According to VCEP guidelines, BA1 requires FAF ≥0.001 in a continental subpopulation. The variant is absent (FAF=0%). Therefore, BA1 is not applied.

BS1

BS1 (Not Applied)

According to VCEP guidelines, BS1 requires FAF ≥0.0003 but <0.001. The variant is absent. Therefore, BS1 is not applied.

BS2

BS2 (Not Applied)

According to VCEP guidelines, BS2 applies when ≥8 healthy older females are observed with the variant. No such observations exist. Therefore, BS2 is not applied.

BS3

BS3 (Not Applied)

According to VCEP guidelines, BS3 requires functional evidence of no LOF in TP53 assays. No functional data exist. Therefore, BS3 is not applied.

BS4

BS4 (Not Applied)

According to VCEP guidelines, BS4 requires lack of segregation in affected family members. No segregation data exist. Therefore, BS4 is not applied.

BP1

BP1 (Not Applied)

According to standard ACMG guidelines, BP1 applies to missense variants in genes where only truncating variants cause disease. This is intronic. Therefore, BP1 is not applied.

BP2

BP2 (Not Applied)

According to standard ACMG guidelines, BP2 applies when in cis with a pathogenic variant or in trans for a dominant disorder. No such data. Therefore, BP2 is not applied.

BP3

BP3 (Not Applied)

According to standard ACMG guidelines, BP3 applies to in-frame indels in repetitive regions. This is a single nucleotide intronic variant. Therefore, BP3 is not applied.

BP4

BP4 (Not Applied)

According to VCEP guidelines, BP4 applies to missense variants with benign in silico scores. This is an intronic variant. Therefore, BP4 is not applied.

BP5

BP5 (Not Applied)

According to standard ACMG guidelines, BP5 applies when a variant is found in trans with a pathogenic variant for a dominant disorder. No such data. Therefore, BP5 is not applied.

BP6

BP6 (Not Applied)

According to standard ACMG guidelines, BP6 applies when a reputable source classifies variant as benign. No such assertion exists. Therefore, BP6 is not applied.

BP7

BP7 (Supporting)

According to VCEP guidelines, 'Supporting BP7: an intronic variant at or beyond +7 to -21 positions with SpliceAI ≤0.1 and no predicted splice impact.' c.-29+121G>T is at +121 and SpliceAI=0. Therefore, BP7 is applied at Supporting strength.