Genetic Information

Gene & Transcript Details

Gene
PALB2
Transcript
NM_024675.4 MANE Select
Total Exons
Reference Sequence
NC_000016.9
Alternative Transcripts
IDStatusDetails
NM_024675.4 MANE Select 4008 nt | 154–3714
NM_024675.3 RefSeq Select 4069 nt | 201–3761

Variant Details

HGVS Notation
NM_024675.4:c.1578T>C
Protein Change
H526=
Location
Exon 4 (Exon 4 of )
4
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 Benign
2 publications
Clinical Statement

"This variant has been reported in ClinVar as Likely benign (2 clinical laboratories) and as Benign (1 clinical laboratories)."

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 PALB2.

Functional Studies & Therapeutic Relevance

Functional Summary

The 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 -45 bp
- Donor Loss (DL) 0.0 -106 bp
+ Acceptor Gain (AG) 0.0 -486 bp
+ Donor Gain (DG) 0.0 -1 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 the rule for PVS1 is: "Use PALB2 PVS1 Decision Tree". The evidence for this variant shows it is a synonymous change and not a null variant. Therefore, this criterion is not applied.

PS1

PS1 (Not Applied)

According to standard ACMG guidelines the rule for PS1 is: "Same amino acid change as a known pathogenic variant regardless of nucleotide change". The evidence for this variant shows no amino acid change. Therefore, this criterion is not applied.

PS2

PS2 (Not Applied)

According to standard ACMG guidelines the rule for PS2 is: "Confirmed de novo in a patient with the disease and no family history". Parental data are not available for this variant. Therefore, this criterion is not applied.

PS3

PS3 (Not Applied)

According to standard ACMG guidelines the rule for PS3 is: "Well-established functional studies show a deleterious effect". No functional studies have been performed for this variant. Therefore, this criterion is not applied.

PS4

PS4 (Not Applied)

According to VCEP guidelines the rule for PS4 is: "Strong case-control studies; p-value ≤ .05 AND (OR ≥ 3 or lower 95% CI ≥ 1.5)". There are no case-control data for this variant. Therefore, this criterion is not applied.

PM1

PM1 (Not Applied)

According to standard ACMG guidelines the rule for PM1 is: "Located in a mutational hot spot or well‐studied functional domain without benign variation". This synonymous variant is not in a known hotspot or functional domain. Therefore, this criterion is not applied.

PM2

PM2 (Supporting)

According to VCEP guidelines the rule for PM2 is: "Supporting – Variant absent in gnomAD or present in ≤ 1/300,000 alleles". The evidence for this variant shows it is not found in gnomAD. Therefore, this criterion is applied at Supporting strength.

PM3

PM3 (Not Applied)

According to VCEP guidelines the rule for PM3 is: "Use Fanconi Anemia PM3 tables" for recessive alleles. This variant is not part of a biallelic context or Fanconi Anemia case. Therefore, this criterion is not applied.

PM4

PM4 (Not Applied)

According to standard ACMG guidelines the rule for PM4 is: "Protein length changes due to in-frame indels or stop-loss variants". This is a synonymous substitution. Therefore, this criterion is not applied.

PM5

PM5 (Not Applied)

According to VCEP guidelines the rule for PM5 is: "Supporting – Apply to frameshifting or truncating variants upstream of p.Tyr1183". This variant is synonymous. Therefore, this criterion is not applied.

PM6

PM6 (Not Applied)

According to standard ACMG guidelines the rule for PM6 is: "Assumed de novo without confirmation of paternity and maternity". No de novo evidence is available. Therefore, this criterion is not applied.

PP1

PP1 (Not Applied)

According to VCEP guidelines the rule for PP1 is: "Segregation LOD thresholds". There are no segregation data. Therefore, this criterion is not applied.

PP2

PP2 (Not Applied)

According to standard ACMG guidelines the rule for PP2 is: "Missense variant in a gene with low rate of benign missense variation". This is a synonymous variant. Therefore, this criterion is not applied.

PP3

PP3 (Not Applied)

According to VCEP guidelines the rule for PP3 is: "Protein: do not use; RNA: at least one predictor shows impact on splicing". SpliceAI predicts no impact on splicing. Therefore, this criterion is not applied.

PP4

PP4 (Not Applied)

According to standard ACMG guidelines the rule for PP4 is: "Patient phenotype or family history highly specific for gene". No phenotype or family history data are provided. Therefore, this criterion is not applied.

PP5

PP5 (Not Applied)

According to standard ACMG guidelines the rule for PP5 is: "Reputable source reports variant as pathogenic". No reputable source reports this variant as pathogenic. Therefore, this criterion is not applied.

BA1

BA1 (Not Applied)

According to VCEP guidelines the rule for BA1 is: "Stand Alone – GnomAD allele frequency > 0.1%". The variant is absent in gnomAD. Therefore, this criterion is not applied.

BS1

BS1 (Not Applied)

According to VCEP guidelines the rule for BS1 is: "Strong – GnomAD allele frequency > 0.01%". The variant is absent in gnomAD. Therefore, this criterion is not applied.

BS2

BS2 (Not Applied)

According to VCEP guidelines the rule for BS2 is: "Use Fanconi Anemia BS2 tables". No relevant data. Therefore, this criterion is not applied.

BS3

BS3 (Not Applied)

According to VCEP guidelines the rule for BS3 is: "Well-established functional studies show no damaging effect". No functional data exist. Therefore, this criterion is not applied.

BS4

BS4 (Not Applied)

According to VCEP guidelines the rule for BS4 is: "Non-segregation in affected/unaffected". No segregation data. Therefore, this criterion is not applied.

BP1

BP1 (Not Applied)

According to VCEP guidelines the rule for BP1 is: "Applies to all missense variants". This is a synonymous variant. Therefore, this criterion is not applied.

BP2

BP2 (Not Applied)

According to standard ACMG guidelines the rule for BP2 is: "Observation in trans with a pathogenic variant for a recessive disorder". No such observations. Therefore, this criterion is not applied.

BP3

BP3 (Not Applied)

According to standard ACMG guidelines the rule for BP3 is: "In-frame deletions/insertions in repetitive region without a known function". Not applicable to a synonymous SNV. Therefore, this criterion is not applied.

BP4

BP4 (Supporting)

According to VCEP guidelines the rule for BP4 is: "RNA: At least one predictor shows no impact on splicing". SpliceAI predicts no splicing impact. Therefore, this criterion is applied at Supporting strength.

BP5

BP5 (Not Applied)

According to standard ACMG guidelines the rule for BP5 is: "Variant found in a case with an alternate molecular basis for disease". No such data. Therefore, this criterion is not applied.

BP6

BP6 (Supporting)

According to standard ACMG guidelines the rule for BP6 is: "Reputable source reports variant as benign but evidence not available". ClinVar entries report this variant as Benign/Likely benign. Therefore, this criterion is applied at Supporting strength.

BP7

BP7 (Not Applied)

According to VCEP guidelines the rule for BP7 is: "Observed lack of aberrant RNA defect for silent substitutions". No RNA assay data are available, only in silico predictions. Therefore, this criterion is not applied.