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Literature summary extracted from

  • Goncalves, S.; Paupe, V.; Dassa, E.P.; Rustin, P.
    Deferiprone targets aconitase: implication for Friedreich's ataxia treatment (2008), BMC Neurol., 8, 20.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
4.2.1.3 medicine enzyme inhibition, e.g. by deferiprone, is helpful in Friedreich's ataxia treatment Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
4.2.1.3 deferiprone the loss of aconitase activity observed in cells should be ascribed to the chelation of available iron rather than to a direct effect of the chelator on the iron-sulfur clusters of the enzyme Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
4.2.1.3 cytosol
-
 Homo sapiens 5829
-
4.2.1.3 mitochondrion mitochondrial aconitase activity represents up to 80% of the total aconitase activity in skin fibroblasts Homo sapiens 5739
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
4.2.1.3 Fe2+ required, the enzyme contains iron-sulfur clusters. Chelating mitochondrial free iron in various cell systems causes loss of aconitase activity Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
4.2.1.3 additional information Homo sapiens impairing aconitase activity precedes decreased cell proliferation ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
4.2.1.3 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.2.1.3 fibroblast
-
 Homo sapiens
-
4.2.1.3 skin
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.2.1.3 additional information impairing aconitase activity precedes decreased cell proliferation Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
4.2.1.3 aconitase
-
 Homo sapiens