About PRKN
No gene-level summary was recorded for this case.
No variant summary was recorded for this case.
Five-step scale from benign to pathogenic. This variant is likely benign. Select a step to read what it means.
Likely benign.A likely benign classification under the recorded framework.
Gene-level background from the recorded interpretation.
Words with a dotted underline have a short definition. Hover or tap them.No gene-level summary was recorded for this case.
The recorded framework is Not recorded. The criteria and strengths shown below come from the saved assessment; this page does not recalculate the classification.
The applied criteria and their strengths are shown as assessed under the stated framework.
No pathogenic criterion marked as applied in the recorded assessment.
This variant has been observed in the homozygous state in population databases: 4 homozygotes in gnomAD v2.1 and 12 homozygotes in gnomAD v4.1. For autosomal recessive early-onset Parkinson disease (ARJP) caused by biallelic PRKN pathogenic variants, the presence of multiple homozygous individuals in general population databases constitutes strong evidence for a benign classification.
Multiple lines of computational evidence suggest no impact on the gene product. BayesDel score 0.144 is in the clearly benign range. SpliceAI predicts no splicing impact (max delta = 0.0). REVEL score 0.627 is in the indeterminate zone but does not reach a pathogenic threshold. Preponderance of in silico evidence supports a benign interpretation.
gene-specific framework lacked a usable explicit final combination framework, so generic ACMG/AMP 2015 final-combination rules were applied as fallback; applied criteria: BS2 strong benign, BP4 supporting benign; combination = 1 strong benign + 1 supporting benign, which maps to Likely Benign.
Evidence summaries and captured source records, where available. Missing entries should not be interpreted as negative findings.
gnomAD v4.1: 2,996 / 1,614,156 alleles. Recorded AF 0.00185608. Recorded homozygote count 12.
This variant is present in gnomAD v2.1 (AF= 0.00186997; MAF= 0.18700%, 529/282892 alleles, homozygotes = 4) and has highest observed frequency in the European (Finnish) population (AF= 0.00338322; MAF= 0.33832%, 85/25124 alleles, homozygotes = 0); grpmax FAF= 0.00214045.
This variant is present in gnomAD-Canada v1.0 (AF= 0.0020086862106406082, 37/18420 alleles, homozygotes = 0).
Recorded SpliceAI maximum delta score: 0.000. Refer to the recorded PP3/BP4 rationale for interpretation.
2 Likely benign · 1 Uncertain significance · 1 Benign. ClinVar API review status: criteria provided, conflicting classifications.
OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. PRKN encodes a tumor suppressor invovled in tagging cellular proteins for degradation. PRKN is inactivated in various cancer types, and its dysfunctio
This variant has previously been reported in somatic cancers (COSMIC; COSV58208986, n = 3 times).
This variant does not lie in a statistically significant hotspot.
ACMG/AMP criteria under Not recorded, with the data and sources behind each one.
26 criteria with recorded statuses: 2 met · 21 not met · 0 not assessed · 3 not applicable. Open any row for the full reasoning, the rule and the evidence behind it.
This variant has been observed in the homozygous state in population databases (4 homozygotes in gnomAD v2.1, 12 homozygotes in gnomAD v4.1), meeting BS2 (strong benign) for an autosomal recessive early-onset Parkinson disease with full penetrance.
Multiple lines of computational evidence suggest no deleterious impact: BayesDel score 0.144 is in the benign range, SpliceAI predicts no splicing effect (max delta 0.0), and REVEL score 0.627 is indeterminate. Meeting BP4 at supporting benign level.
Combination of BS2 (strong benign) and BP4 (supporting benign) meets the generic ACMG/AMP 2015 threshold for Likely Benign (1 Strong benign + 1 Supporting benign).
No criteria match.
Every interpreted variant in PRKN, mapped onto the exon structure of NM_004562.2. Select a row to find it on the plot.
| Protein | Location | Classification | Link |
|---|
| Middle Eastern23 / 6,060 | 0.00381 hom |
| European (Finnish)197 / 64,036 | 0.0031 |
| South Asian187 / 91,086 | 0.00214 hom |
| Admixed American121 / 60,032 | 0.002 |
| European (non-Finnish)2286 / 1,180,002 | 0.00197 hom |
| Remaining individuals111 / 62,506 | 0.0018 |
| African/African American67 / 75,044 | 0.00089 |
| Ashkenazi Jewish3 / 29,606 | 0.0001 |
| East Asian1 / 44,874 | 2.2e-05 |
| European (Finnish)85 / 25,124 | 0.0034 |
| Remaining individuals17 / 7,226 | 0.0024 |
| European (non-Finnish)290 / 129,198 | 0.00222 hom |
| South Asian58 / 30,616 | 0.00192 hom |
| Admixed American52 / 35,440 | 0.0015 |
| African/African American27 / 24,966 | 0.0011 |
This variant is present in gnomAD v4.1 (AF= 0.00185608; MAF= 0.18561%, 2996/1614156 alleles, homozygotes = 12) and has highest observed frequency in the Middle Eastern population (AF= 0.00379538; MAF= 0.37954%, 23/6060 alleles, homozygotes = 1); grpmax FAF= 0.00259375.
This variant is present in gnomAD v2.1 (AF= 0.00186997; MAF= 0.18700%, 529/282892 alleles, homozygotes = 4) and has highest observed frequency in the European (Finnish) population (AF= 0.00338322; MAF= 0.33832%, 85/25124 alleles, homozygotes = 0); grpmax FAF= 0.00214045.
This variant is present in gnomAD-Canada v1.0 (AF= 0.0020086862106406082, 37/18420 alleles, homozygotes = 0).
SpliceAI predicts no significant splice impact for this variant (max delta score = 0.00). REVEL score = 0.627. BayesDel score = 0.143758.
5 paper records listed. No publication is explicitly linked to an applied criterion in the recorded metadata. None of the listed paper records explicitly indicates that the variant is named.
Genetic testing for specific mutations is recommended on an individual basis (Level B), taking into account specific features (i.e. family history and age of onset).
Abstract reviewed; full-text not available
SYNJ1 has been recently identified by two independent groups as the gene defective in a novel form of autosomal recessive, early-onset atypical parkinsonism (PARK20).
Abstract reviewed; full-text not available
We undertook an iterative process of refining the ACMG-AMP guidelines. We used the guidelines to classify more than 40,000 clinically observed variants, assessed the outcome, and refined the classification criteria to capture exceptions and edge cases.
Abstract reviewed; full-text not available
Screenshots represent source records captured during evidence collection. Select one to view the recorded source content.
The evidence statements recorded by the interpretation pipeline. They have not been reinterpreted by the page.
This variant is present in gnomAD v4.1 (AF= 0.00185608; MAF= 0.18561%, 2996/1614156 alleles, homozygotes = 12) and has highest observed frequency in the Middle Eastern population (AF= 0.00379538; MAF= 0.37954%, 23/6060 alleles, homozygotes = 1); grpmax FAF= 0.00259375.
This variant is present in gnomAD v2.1 (AF= 0.00186997; MAF= 0.18700%, 529/282892 alleles, homozygotes = 4) and has highest observed frequency in the European (Finnish) population (AF= 0.00338322; MAF= 0.33832%, 85/25124 alleles, homozygotes = 0); grpmax FAF= 0.00214045.
This variant is present in gnomAD-Canada v1.0 (AF= 0.0020086862106406082, 37/18420 alleles, homozygotes = 0).
This variant has been reported in ClinVar as Likely benign (2 clinical laboratories) and as Uncertain significance (1 clinical laboratory) and as Benign (1 clinical laboratory). (ClinVarID = 425402)
SpliceAI predicts no significant splice impact for this variant (max delta score = 0.00). REVEL score = 0.627. BayesDel score = 0.143758.
OncoKB did not identify variant-specific reviewed functional evidence for this variant; gene-level curated context is available for reviewer follow-up. PRKN encodes a tumor suppressor invovled in tagging cellular proteins for degradation. PRKN is inactivated in various cancer types, and its dysfunctio
This variant does not lie in a statistically significant hotspot. This variant has previously been reported in somatic cancers (COSMIC; COSV58208986, n = 3 times).
This variant does not lie in a statistically significant hotspot.