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Details on Person UniProt:P00441 SOD1
| Class:Id | ReferenceGeneProduct:64824 |
|---|---|
| _chainChangeLog | initiator methionine:1 added on Sat February 7 2015;chain:2-154 added on Sat February 7 2015;initiator methionine:1 for 64824 removed on Fri Nov 03 2023;initiator methionine: for 64824 added on Fri Nov 03 2023;initiator methionine: for 64824 removed on Fri Aug 15 2025;initiator methionine:1 for 64824 added on Fri Aug 15 2025 |
| _displayName | UniProt:P00441 SOD1 |
| _timestamp | 2026-02-20 22:16:17 |
| chain | initiator methionine:1 chain:2-154 |
| checksum | 25CA38DA8D564483 |
| comment | FUNCTION Destroys radicals which are normally produced within the cells and which are toxic to biological systems (PubMed:24140062). Catalyzes the oxidation of hydrogen sulfide (H2S) to sulfate, playing an important role in detoxifying H2S and limiting the accumulation of reactive sulfur species (RSS) such as persulfides and polysulfides (PubMed:36630448).CATALYTIC ACTIVITY 2 superoxide + 2 H(+) = H2O2 + O2CATALYTIC ACTIVITY hydrogen sulfide + 2 O2 = sulfate + H(+)COFACTOR Binds 1 copper ion per subunit.COFACTOR Binds 1 zinc ion per subunit.SUBUNIT Homodimer; non-disulfide-linked (By similarity). Homodimerization may take place via the ditryptophan cross-link at Trp-33. Heterodimer with SOD1 (PubMed:31292775). The heterodimer CCS:SOD1 interacts with SLC31A1; this heterotrimer is Cu(1+)-mediated and its maintenance is regulated through SOD1 activation (PubMed:31292775). Interacts with DAOA; the interaction is direct (PubMed:30037290).INTERACTION Predominantly cytoplasmic; the pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.PTM Unlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation.PTM The ditryptophan cross-link at Trp-33 is responsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required.PTM Palmitoylation helps nuclear targeting and decreases catalytic activity.PTM Succinylation, adjacent to copper catalytic site, probably inhibits activity. Desuccinylation by SIRT5 enhances activity.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS The protein (both wild-type and ALS1 variants) has a tendency to form fibrillar aggregates in the absence of the intramolecular disulfide bond or of bound zinc ions. These aggregates may have cytotoxic effects. Zinc binding promotes dimerization and stabilizes the native form.SIMILARITY Belongs to the Cu-Zn superoxide dismutase family.SEQUENCE CAUTION ALS genetic mutations dbONLINE INFORMATION Superoxide dismutase entry |
| description | recommendedName: fullName evidence="81"Superoxide dismutase [Cu-Zn] ecNumber evidence="35"1.15.1.1 ecNumber evidence="81"1.8.-.- alternativeName: fullName evidence="80"Hydrogen sulfide oxidase alternativeName: Superoxide dismutase 1 shortName: hSod1 |
| geneName | SOD1 |
| identifier | P00441 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Amyotrophic lateral sclerosis Antioxidant Copper Cytoplasm Direct protein sequencing Disease variant Disulfide bond Lipoprotein Metal-binding Neurodegeneration Nucleus Oxidoreductase Palmitate Phosphoprotein Proteomics identification Reference proteome Ubl conjugation Zinc |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9862192] Weiser, Joel, 2024-02-26 [InstanceEdit:9909836] Weiser, Joel, 2024-05-14 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9963647] Weiser, Joel, 2025-08-15 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | SOD1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8950613] ENSEMBL:ENSG00000142168 SOD1 [Homo sapiens] |
| secondaryIdentifier | SODC_HUMAN A6NHJ0 D3DSE4 Q16669 Q16711 Q16838 Q16839 Q16840 Q6NR85 |
| sequenceLength | 154 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:350697] SOD1 [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:350705] SOD1 [extracellular region] [Homo sapiens] [EntityWithAccessionedSequence:3299726] 2xHC-SOD1 [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:3777082] SOD1 [mitochondrial intermembrane space] [Homo sapiens] [EntityWithAccessionedSequence:3777108] 2xHC-SOD1 [mitochondrial intermembrane space] [Homo sapiens] |
| (referenceSequence) | [IntraChainCrosslinkedResidue:3299696] Intra-chain Crosslink via L-cystine (cross-link) at 57 and 146 |
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No pathways have been reviewed or authored by UniProt:P00441 SOD1 (64824)
