ADPRS

Chr 1AR

ADP-ribosylserine hydrolase

Also known as: ADPRHL2, ARH3, CONDSIAS

The protein is an ADP-ribosylhydrolase that removes ADP-ribose modifications from proteins and degrades free poly(ADP-ribose), playing a key role in DNA damage response and protecting cells from poly(ADP-ribose)-dependent cell death. Mutations cause autosomal recessive neurodegeneration with childhood onset that is stress-induced and features variable ataxia and seizures. The gene shows low constraint to loss-of-function variants (pLI 0.0006, LOEUF 1.03).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.031 OMIM phenotype
Clinical SummaryADPRS
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.03LOEUF
pLI 0.001
Z-score 1.49
OE 0.55 (0.311.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.35Z-score
OE missense 0.93 (0.821.05)
186 obs / 199.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.55 (0.311.03)
00.351.4
Missense OE0.93 (0.821.05)
00.61.4
Synonymous OE1.15
01.21.6
LoF obs/exp: 7 / 12.7Missense obs/exp: 186 / 199.9Syn Z: -1.07
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongADPRS-related neurodegeneration with developmental delay ataxia and axonal neuropathyLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.5968th %ile
GOF
0.6344th %ile
LOF
0.2872th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ADPRS · 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.

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Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗