PARK7

Chr 1AR

Parkinsonism associated deglycase

Also known as: DJ-1, DJ1, GATD2, HEL-S-67p

The protein functions as a redox-sensitive chaperone and protease that protects neurons against oxidative stress and cell death, and maintains correct mitochondrial morphology and function. Mutations cause autosomal recessive early-onset Parkinson disease 7, typically manifesting in childhood or young adulthood. The gene shows moderate constraint to loss-of-function variants (pLI 0.75, LOEUF 0.53), consistent with its essential neuroprotective role.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Parkinson disease 7, autosomal recessive early-onsetMIM #606324
AR
0
Active trials
77
Pubs (1 yr)
72
P/LP submissions
15%
P/LP missense
0.53
LOEUF
Mechanism
Clinical SummaryPARK7
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Gene-Disease Validity (ClinGen)
Parkinson disease · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.75) — some intolerance to loss-of-function variants.
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ClinVar Variants
61 unique Pathogenic / Likely Pathogenic· 62 VUS of 214 total submissions
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GeneReview available — PARK7
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.53LOEUF
pLI 0.753
Z-score 2.47
OE 0.11 (0.040.53)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.16Z-score
OE missense 0.96 (0.811.13)
104 obs / 108.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.11 (0.040.53)
00.351.4
Missense OE0.96 (0.811.13)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 1 / 9.0Missense obs/exp: 104 / 108.8Syn Z: 0.34

ClinVar Variant Classifications

214 submitted variants in ClinVar

Classification Summary

Pathogenic54
Likely Pathogenic7
VUS62
Likely Benign35
Benign41
Conflicting1
54
Pathogenic
7
Likely Pathogenic
62
VUS
35
Likely Benign
41
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
6
46
0
54
Likely Pathogenic
3
3
1
0
7
VUS
3
44
15
0
62
Likely Benign
0
3
22
10
35
Benign
0
0
41
0
41
Conflicting
1
Total85612510200

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PARK7 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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