Protein Information

Name aconitase
Synonyms ACO 2; Aconitase; Citrate hydro lyase; ACO2; ACO2 protein; ACONM; Aconitase 2; Citrate hydrolyase aconitase…

Compound Information

Name sulfur
CAS sulfur

Reference List

PubMed Abstract RScore(About this table)
19865480 Xu XM, Lin H, Latijnhouwers M, Moller SG: Dual localized AtHscB involved in iron sulfur protein biogenesis in Arabidopsis. PLoS One. 2009 Oct 29;4(10):e7662.

Furthermore, AtHscB is highly expressed in anthers and trichomes and an AtHscB T-DNA insertion mutant shows reduced seed set, a waxless phenotype and inappropriate trichome development as well as dramatically reduced activities of the iron-sulfur enzymes aconitase and succinate dehydrogenase.
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19864422 Song D, Tu Z, Lee FS: Human ISCA1 interacts with IOP1/NARFL and functions in both cytosolic and mitochondrial iron-sulfur protein biogenesis. J Biol Chem. 2009 Dec 18;284(51):35297-307. Epub .

We observe that small interfering RNA knockdown of IscA1 in HeLa cells leads to decreased activity of two mitochondrial iron-sulfur enzymes, succinate dehydrogenase and mitochondrial aconitase, as well as a cytosolic iron-sulfur enzyme, cytosolic aconitase.
31(0,1,1,1) Details
19567699 Kollberg G, Tulinius M, Melberg A, Darin N, Andersen O, Holmgren D, Oldfors A, Holme E: Clinical manifestation and a new ISCU mutation in iron-sulphur cluster deficiency myopathy. Brain. 2009 Aug;132(Pt 8):2170-9. Epub 2009 Jun 30.


Myopathy with deficiency of succinate dehydrogenase and aconitase is a recessively inherited disorder characterized by childhood-onset early fatigue, dyspnoea and palpitations on trivial exercise.
1(0,0,0,1) Details
19347027 Schulz JB, Boesch S, Burk K, Durr A, Giunti P, Mariotti C, Pousset F, Schols L, Vankan P, Pandolfo M: Diagnosis and treatment of Friedreich ataxia: a European perspective. Nat Rev Neurol. 2009 Apr;5(4):222-34.

FXN mutations cause deficiencies of the iron-sulfur cluster-containing subunits of the mitochondrial electron transport complexes I, II, and III, and of the iron-sulfur protein aconitase.
31(0,1,1,1) Details
19520720 van Vugt-Lussenburg BM, van der Weel L, Hagen WR, Hagedoorn PL: Identification of two [4Fe-4S]-cluster-containing hydro-lyases from Pyrococcus furiosus. Microbiology. 2009 Sep;155(Pt 9):3015-20. Epub 2009 Jun 11.


Nonetheless, its genome encodes more putative TCA cycle enzymes than the closely related Pyrococcus horikoshii and Pyrococcus abyssi, including an aconitase (PF0201).
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19453295 Metzendorf C, Wu W, Lind MI: Overexpression of Drosophila mitoferrin in l (2) mbn cells results in dysregulation of Fer1HCH expression. Biochem J. 2009 Jul 15;421(3):463-71.


Overexpression of dmfrn in the Drosophila l (2) mbn cell line (mbn-dmfrn) resulted in decreased binding between IRP-1A (iron regulatory protein 1A) and stem-loop RNA structures referred to as IREs (iron responsive elements). mbn-dmfrn cell lines also had increased cytoplasmic aconitase activity and slightly decreased iron content.
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19383690 Calderon IL, Elias AO, Fuentes EL, Pradenas GA, Castro ME, Arenas FA, Perez JM, Vasquez CC: Tellurite-mediated disabling of [4Fe-4S] clusters of Escherichia coli dehydratases. Microbiology. 2009 Jun;155(Pt 6):1840-6. Epub 2009 Apr 21.


Reactive oxygen species (ROS)-sensitive fumarase A (FumA) and aconitase B (AcnB) as well as ROS-resistant fumarase C (FumC) and aconitase A (AcnA) were assayed in cell-free extracts from tellurite-exposed cells in both the presence and absence of oxygen.
1(0,0,0,1) Details
19710232 Bernard DG, Cheng Y, Zhao Y, Balk J: An allelic mutant series of ATM3 reveals its key role in the biogenesis of cytosolic iron-sulfur proteins in Arabidopsis. Plant Physiol. 2009 Oct;151(2):590-602. Epub 2009 Aug 26.

Analyses of selected metal enzymes showed that the activity of cytosolic aconitase (Fe-S) was strongly decreased across the range of atm3 alleles, whereas mitochondrial and plastid Fe-S enzymes were unaffected.
1(0,0,0,1) Details
19808020 Chan SY, Zhang YY, Hemann C, Mahoney CE, Zweier JL, Loscalzo J: MicroRNA-210 controls mitochondrial metabolism during hypoxia by repressing the iron-sulfur cluster assembly proteins ISCU1/2. Cell Metab. 2009 Oct;10(4):273-84.

In turn, by repressing ISCU1/2 during hypoxia, miR-210 decreases the activity of prototypical iron-sulfur proteins controlling mitochondrial metabolism, including Complex I and aconitase.
1(0,0,0,1) Details
19492851 Kim JH, Fuzery AK, Tonelli M, Ta DT, Westler WM, Vickery LE, Markley JL: Structure and dynamics of the iron-sulfur cluster assembly scaffold protein IscU and its interaction with the cochaperone HscB. Biochemistry. 2009 Jul 7;48(26):6062-71.

Its critical importance has been recently underscored by the finding that a single intronic mutation in the human iscu gene is associated with a myopathy resulting from deficient succinate dehydrogenase and aconitase [Mochel, F., Knight, M.
1(0,0,0,1) Details
19397952 Ma YS, Wu SB, Lee WY, Cheng JS, Wei YH: Response to the increase of oxidative stress and mutation of mitochondrial DNA in aging. Biochim Biophys Acta. 2009 Oct;1790(10):1021-9. Epub 2009 May 4.

Recently, we observed that gene expression of several proteins and enzymes related to iron metabolism is altered and that aconitase is extremely susceptible to oxidative damage in senescent skin fibroblasts and in cybrids harboring aging-associated A8344G mutation of mtDNA.
Of great importance is the perturbation at the protein and activity levels of several enzymes containing iron-sulfur clusters in skin fibroblasts of elderly subjects.
1(0,0,0,1) Details
20080550 Grawert T, Span I, Eisenreich W, Rohdich F, Eppinger J, Bacher A, Groll M: Probing the reaction mechanism of IspH protein by x-ray structure analysis. Proc Natl Acad Sci U S A. 2010 Jan 19;107(3):1077-81. Epub 2009 Dec 28.

This conversion is catalyzed by IspH protein comprising a central iron-sulfur cluster as electron transfer cofactor in the active site.
The IspH:substrate complex reveals a hairpin conformation of the ligand with the C (1) hydroxyl group coordinated to the unique site in a [4Fe-4S] cluster of aconitase type.
1(0,0,0,1) Details
20053667 Condo I, Malisan F, Guccini I, Serio D, Rufini A, Testi R: Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin. Hum Mol Genet. 2010 Apr 1;19(7):1221-9. Epub 2010 Jan 6.

We demonstrate that the extramitochondrial form of frataxin directly interacts with cytosolic aconitase/iron regulatory protein-1 (IRP1), a bifunctional protein alternating between an enzymatic and a RNA-binding function through the 'iron-sulfur switch' mechanism.
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19348893 Amutha B, Gordon DM, Dancis A, Pain D: Chapter 14 Nucleotide-dependent iron-sulfur cluster biogenesis of endogenous and imported apoproteins in isolated intact mitochondria. Methods Enzymol. 2009;456:247-66.

Both endogenous (Aco1p aconitase) and newly imported (Yah1p ferredoxin) apoproteins can be used as substrates. [Fe-S] cluster biogenesis in isolated intact mitochondria is greatly enhanced by the addition of nucleotides (GTP and ATP) and requires hydrolysis of both.
1(0,0,0,1) Details
20097860 Albrecht AG, Netz DJ, Miethke M, Pierik AJ, Burghaus O, Peuckert F, Lill R, Marahiel MA: SufU is an essential iron-sulfur cluster scaffold protein in Bacillus subtilis. J Bacteriol. 2010 Mar;192(6):1643-51. Epub 2010 Jan 22.

Bacteria use three distinct systems for iron-sulfur (Fe/S) cluster biogenesis: the ISC, SUF, and NIF machineries.
The drastic growth retardation of a conditional mutant depleted of SufU was coupled with a severe reduction of aconitase and succinate dehydrogenase activities in total-cell lysates, suggesting a crucial function of SufU in Fe/S protein biogenesis.
1(0,0,0,1) Details
20113483 Pieper R, Huang ST, Parmar PP, Clark DJ, Alami H, Fleischmann RD, Perry RD, Peterson SN: Proteomic analysis of iron acquisition, metabolic and regulatory responses of Yersinia pestis to iron starvation. BMC Microbiol. 2010 Jan 29;10:30.

The iron-sulfur (Fe-S) cluster assembly system Suf, adapted to oxidative stress and iron starvation in E. coli, was also more abundant, suggesting functional activity of Suf in Y. pestis under iron-limiting conditions.
This data was consistent with lower activities of aconitase and catalase in iron-starved vs. iron-rich cells.
1(0,0,0,1) Details
19884169 Leidgens S, De Smet S, Foury F: Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet. Hum Mol Genet. 2010 Jan 15;19(2):276-86. Epub 2009 Nov 2.

Friedreich's ataxia is a neurodegenerative disease caused by the low expression of frataxin, a mitochondrial iron-binding protein which plays an important, but non-essential, role in the formation of iron-sulfur (Fe/S) clusters.
The Q129A, I130A, W131A (F) and R141A mutations, which reside in surface exposed residues of the fourth and fifth beta-strands, result in severe cell growth inhibition on high-iron media and low aconitase activity, indicating that Fe/S cluster biosynthesis is impaired.
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