| Gene Symbol | ACO2 |
| Full Name | Aconitase 2 |
| Chromosome | 22q13.2 |
| Protein Type | Enzyme |
| Function | is a mitochondrial enzyme that catalyzes the stereospecific isomerization of citrate to isocitrate via cis-aconitate in the second step of the tricarboxylic acid (TCA) cycle[@beinert1996]. |
| Primary Expression | all nucleated cells: |
| Amino Acids | 778 aa |
| Exons | 18 |
| Ensembl ID | ENSG00000100412 |
| GeneCards | ACO2 |
| Human Protein Atlas | ACO2 |
| Four functional domains | Organized around the 4Fe-4S cluster |
| 4Fe-4S cluster binding site | Essential for catalytic activity and redox sensing |
| Active site | Contains three conserved cysteine residues coordinating the iron-sulfur cluster |
| Citrate → cis-Aconitate → Isocitrate | Stereochemical isomerization |
| Associated Diseases | neurodegeneration |
| Interactions | NDUFS1, NDUFS2, CYCS, HSPA9, SDHA, SNCA |
| KG Connections | 81 knowledge graph edges |
| Databases | GeneCardsUniProtNCBI GeneHPASTRING |