ACO2 isomerizes citrate

Stable Identifier
R-HSA-70971
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
citrate => D-threo-isocitrate
ReviewStatus
5/5
Locations in the PathwayBrowser
General
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Mitochondrial aconitase (ACO2) reversibly converts citrate to isocitrate via a cis-aconitate intermediate. Mitochondrial aconitase activity has been demonstrated in diverse human tissue extracts (Slaughter et al., 1975), and a protein homologous to the well-characterized porcine enzyme has been purified from human tissues (Baldwin et al., 1991). Mutations in the ACO2 gene can lead to an infantile neurodegenerative disease (ICRD; MIM:614559) and optic atrophy (OPA9; MIM:616289) (see Guehlouz et al., 2021).
Literature References
PubMed ID Title Journal Year
34354088 ACO2 clinicobiological dataset with extensive phenotype ontology annotation

Desquiret-Dumas, V, Milea, D, Procaccio, V, den Dunnen, JT, Bris, C, Reynier, P, Amati-Bonneau, P, Colin, E, Guehlouz, K, Foulonneau, T, Lenaers, G, Ferré, M, Bonneau, D, Charif, M, Gohier, P

Sci Data 2021
1946331 Purification and partial amino acid sequence of human aconitase

Baldwin, GS, Moritz, RL, Toh, BH, Simpson, R, Callaghan, J, Seet, KL, Toncich, G, Rubira, MR

Protein Seq Data Anal 1991
1052766 Aconitase polymorphism in man

Hopkinson, DA, Harris, H, Slaughter, CA

Ann Hum Genet 1975
Participants
Participates
Catalyst Activity

aconitate hydratase activity of ACO2:4Fe-4S [mitochondrial matrix]

This event is regulated
Orthologous Events
Cross References
Rhea
RHEA
Reviewed
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