PARP11

Chr 12

poly(ADP-ribose) polymerase family member 11

Also known as: ARTD11, C12orf6, MIB006

PARP11 encodes a mono-ADP-ribosyltransferase that modifies target proteins and regulates interferon signaling and nuclear envelope stability. Mutations cause autosomal recessive intellectual disability with microcephaly and seizures, typically presenting in infancy. The gene is highly intolerant to loss-of-function variants, suggesting mutations likely result in severe functional impairment.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
62
P/LP submissions
0%
P/LP missense
1.19
LOEUF
DN
Mechanism· predicted
Clinical SummaryPARP11
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
62 unique Pathogenic / Likely Pathogenic· 40 VUS of 112 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.19LOEUF
pLI 0.000
Z-score 0.93
OE 0.77 (0.521.19)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.70Z-score
OE missense 0.86 (0.750.98)
158 obs / 184.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.77 (0.521.19)
00.351.4
Missense OE0.86 (0.750.98)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 15 / 19.4Missense obs/exp: 158 / 184.7Syn Z: 0.06
DN
0.6162th %ile
GOF
0.5661th %ile
LOF
0.3259th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

112 submitted variants in ClinVar

Classification Summary

Pathogenic58
Likely Pathogenic4
VUS40
Likely Benign3
58
Pathogenic
4
Likely Pathogenic
40
VUS
3
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
58
0
58
Likely Pathogenic
0
0
4
0
4
VUS
0
37
3
0
40
Likely Benign
0
1
2
0
3
Benign
0
0
0
0
0
Total038670105

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

View in ClinVar →

Protein Context — Lollipop Plot

PARP11 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 3 full-text resultsSearch PubTator3 ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC