Genetic Information

Gene & Transcript Details

Gene
PMS2
Transcript
NM_000535.7 MANE Select
Total Exons
Reference Sequence
NC_000007.13
Alternative Transcripts
IDStatusDetails
NM_000535.6 Alternative 5156 nt | 88–2676
NM_000535.4 Alternative 2836 nt | 88–2676
NM_000535.7 MANE Select 5093 nt | 31–2619
NM_000535.3 Alternative 2820 nt | 57–2645
NM_000535.5 Alternative 2851 nt | 88–2676

Variant Details

HGVS Notation
NM_000535.7:c.23+32dup
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
0.000799 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 Benign
1 publications
Clinical Statement

"This variant has been reported in ClinVar as Likely benign (1 clinical laboratories)."

COSMIC Somatic Evidence

Open
COSMIC ID
COSN17132638
Recurrence
1 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 PMS2.

Functional Studies & Therapeutic Relevance

Functional Summary

Error in OpenAI Consolidation. OncoKB: PMS223+32dupPMS223+32dupSomaticNCBI Gene:5395|Show additional gene information Variant OverviewPMS2, an endonuclease involved in DNA repair, is altered in various cancers.The PMS2 23+32dup alteration 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
Unknown
PP3 Applied
No
REVEL Score
0.0
Threshold: ≥0.75 = PP3 applied

SpliceAI Scores

Window: ±500bp
Effect Type Score Position
- Acceptor Loss (AL) 0.02 -381 bp
- Donor Loss (DL) 0.01 -157 bp
+ Acceptor Gain (AG) 0.0 -354 bp
+ Donor Gain (DG) 0.01 -139 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:
PM2

PM2 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)

BP4

BP4 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)

BP6

BP6 (Unknown (Pre-LLM))

From pre-LLM assessment (LLM Failed)