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Literature summary for 4.2.1.3 extracted from

  • Condo, I.; Malisan, F.; Guccini, I.; Serio, D.; Rufini, A.; Testi, R.
    Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin (2010), Hum. Mol. Genet., 19, 1221-1229.
    View publication on PubMed

Application

Application Comment Organism
medicine it is shown that the cytosolic aconitase defect and consequent IRP1 activation occurring in Friedreich's Ataxia (FRDA) cells are reversed by the action of extramitochondrial frataxin Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens it is demonstrated that the extramitochondrial form of frataxin directly interacts with cytosolic aconitase/iron regulatory protein-1 (IRP1). The inability to produce normal levels of the mitochondrial protein frataxin causes the hereditary degenerative disorder Friedreich’s Ataxia (FRDA) ?
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?

Organism

Organism UniProt Comment Textmining
Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information it is demonstrated that the extramitochondrial form of frataxin directly interacts with cytosolic aconitase/iron regulatory protein-1 (IRP1). The inability to produce normal levels of the mitochondrial protein frataxin causes the hereditary degenerative disorder Friedreich’s Ataxia (FRDA) Homo sapiens ?
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?

Synonyms

Synonyms Comment Organism
aconitase
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Homo sapiens