Reactome | ACO2 isomerizes citrate

ACO2 isomerizes citrate

Stable Identifier

R-HSA-70971

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

citrate => D-threo-isocitrate

ReviewStatus

5/5

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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 Guehlouz, K, Foulonneau, T, Amati-Bonneau, P, Charif, M, Colin, E, Bris, C, Desquiret-Dumas, V, Milea, D, Gohier, P, Procaccio, V, Bonneau, D, den Dunnen, JT, Lenaers, G, Reynier, P, Ferré, M	Sci Data	2021
1946331	Purification and partial amino acid sequence of human aconitase Baldwin, GS, Seet, KL, Callaghan, J, Toncich, G, Toh, BH, Moritz, RL, Rubira, MR, Simpson, R	Protein Seq Data Anal	1991
1052766	Aconitase polymorphism in man Slaughter, CA, Hopkinson, DA, Harris, H	Ann Hum Genet	1975