Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
3.2.1.143
Hydrolases;
Glycosylases;
Glycosidases, i.e. enzymes that hydrolyse O- and S-glycosyl compounds;
poly(ADP-ribose) glycohydrolase
3.2.2.-
Hydrolases;
Glycosylases;
Hydrolysing N-glycosyl compounds;
3.5.1.-
Hydrolases;
Acting on carbon-nitrogen bonds, other than peptide bonds;
In linear amides;
PDB | Resolution (Å) | PDB name |
---|---|---|
2FOZ | 1.6 | human ADP-ribosylhydrolase 3 |
2FP0 | 2.05 | human ADP-ribosylhydrolase 3 |
2G4K | 1.82 | Anomalous substructure of human ADP-ribosylhydrolase 3 |
5ZQY | 1.577 | Crystal structure of a poly(ADP-ribose) glycohydrolase |
6D36 | 1.7 | Structure of human ARH3 bound to ADP-ribose and magnesium |
6D3A | 1.60001 | Structure of human ARH3 D314E bound to ADP-ribose and magnesium |
7AKR | 1.95 | Human ADP-ribosylserine hydrolase ARH3 mutant E41A in complex with ADP-ribose dimer |
7AKS | 1.86 | Human ADP-ribosylserine hydrolase ARH3 mutant E41A in complex with H2B-S7-mar peptide |
7ARW | 1.31 | Structure of human ARH3 E41A bound to alpha-NAD+ and magnesium |
7L9F | 1.75 | Crystal structure of human ARH3 bound to calcium and ADP-ribose |
7L9H | 1.85 | Crystal structure of human ARH3-D77A bound to magnesium and ADP-ribose |
7L9I | 1.8 | Crystal structure of human ARH3-D314A bound to magnesium and ADP-ribose |
GO ontology | GO term | GO description |
---|---|---|
Biological Process | GO:0006281 | DNA repair |
Biological Process | GO:0006287 | base-excision repair, gap-filling |
Biological Process | GO:0071451 | cellular response to superoxide |
Biological Process | GO:0060546 | negative regulation of necroptotic process |
Biological Process | GO:0140290 | peptidyl-serine ADP-deribosylation |
Molecular Function | GO:0140292 | ADP-ribosylserine hydrolase activity |
Molecular Function | GO:0061463 | O-acetyl-ADP-ribose deacetylase activity |
Molecular Function | GO:0004553 | hydrolase activity, hydrolyzing O-glycosyl compounds |
Molecular Function | GO:0000287 | magnesium ion binding |
Molecular Function | GO:0004649 | poly(ADP-ribose) glycohydrolase activity |
Cellular Component | GO:0005737 | cytoplasm |
Cellular Component | GO:0005759 | mitochondrial matrix |
Cellular Component | GO:0005739 | mitochondrion |
Cellular Component | GO:0016604 | nuclear body |
Cellular Component | GO:0005654 | nucleoplasm |
Cellular Component | GO:0005634 | nucleus |
Cellular Component | GO:0090734 | site of DNA damage |
Reactome | Reactome Name | Node type | Reactome Root | Reactome Root Name |
---|---|---|---|---|
R-HSA-110362 | POLB-Dependent Long Patch Base Excision Repair | Leaf | R-HSA-73894 | DNA Repair |
Location | ECO term | Pubmed |
---|---|---|
Chromosome | ECO:0000269 | PubMed:30045870 |
Cytoplasm | ECO:0000269 | PubMed:16278211 |
Cytoplasm | ECO:0000269 | PubMed:34479984 |
Mitochondrion matrix | ECO:0000305 | PubMed:17991898 |
Mitochondrion matrix | ECO:0000305 | PubMed:22433848 |
Mitochondrion matrix | ECO:0000305 | PubMed:34479984 |
Nucleus | ECO:0000269 | PubMed:17991898 |
Nucleus | ECO:0000269 | PubMed:34479984 |
HPO ID | HPO name |
---|---|
HP:0000007 | Autosomal recessive inheritance |
HP:0000252 | Microcephaly |
HP:0000407 | Sensorineural hearing impairment |
HP:0000486 | Strabismus |
HP:0000508 | Ptosis |
HP:0000602 | Ophthalmoplegia |
HP:0000639 | Nystagmus |
HP:0000651 | Diplopia |
HP:0001250 | Seizure |
HP:0001251 | Ataxia |
HP:0001260 | Dysarthria |
HP:0001263 | Global developmental delay |
HP:0001272 | Cerebellar atrophy |
HP:0001308 | Tongue fasciculations |
HP:0001310 | Dysmetria |
HP:0001324 | Muscle weakness |
HP:0001761 | Pes cavus |
HP:0002059 | Cerebral atrophy |
HP:0002069 | Bilateral tonic-clonic seizure |
HP:0002080 | Intention tremor |
HP:0002093 | Respiratory insufficiency |
HP:0002121 | Generalized non-motor (absence) seizure |
HP:0002376 | Developmental regression |
HP:0002465 | Poor speech |
HP:0003447 | Axonal loss |
HP:0003487 | Babinski sign |
HP:0006855 | Cerebellar vermis atrophy |
HP:0011463 | Childhood onset |
HP:0031165 | Multifocal seizures |
Disease name | MONDO ID | ICD10 | ICD10 chapter | OMIM | Orphanet |
---|---|---|---|---|---|
neurodegeneration- childhood-onset- stress-induced- with variable ataxia and seizures | MONDO:0100095 | G31 | chapter6, Diseases of the nervous system | OMIM:618170 |