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Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity.
Science. 2004 Jul 09; 305(5681):242-5.Sci

Abstract

Numerous degenerative disorders are associated with elevated levels of prooxidants and declines in mitochondrial aconitase activity. Deficiency in the mitochondrial iron-binding protein frataxin results in diminished activity of various mitochondrial iron-sulfur proteins including aconitase. We found that aconitase can undergo reversible citrate-dependent modulation in activity in response to pro-oxidants. Frataxin interacted with aconitase in a citrate-dependent fashion, reduced the level of oxidant-induced inactivation, and converted inactive [3Fe-4S]1+ enzyme to the active [4Fe-4S]2+ form of the protein. Thus, frataxin is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation.

Authors+Show Affiliations

Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

15247478

Citation

Bulteau, Anne-Laure, et al. "Frataxin Acts as an Iron Chaperone Protein to Modulate Mitochondrial Aconitase Activity." Science (New York, N.Y.), vol. 305, no. 5681, 2004, pp. 242-5.
Bulteau AL, O'Neill HA, Kennedy MC, et al. Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science. 2004;305(5681):242-5.
Bulteau, A. L., O'Neill, H. A., Kennedy, M. C., Ikeda-Saito, M., Isaya, G., & Szweda, L. I. (2004). Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science (New York, N.Y.), 305(5681), 242-5.
Bulteau AL, et al. Frataxin Acts as an Iron Chaperone Protein to Modulate Mitochondrial Aconitase Activity. Science. 2004 Jul 9;305(5681):242-5. PubMed PMID: 15247478.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. AU - Bulteau,Anne-Laure, AU - O'Neill,Heather A, AU - Kennedy,Mary Claire, AU - Ikeda-Saito,Masao, AU - Isaya,Grazia, AU - Szweda,Luke I, PY - 2004/7/13/pubmed PY - 2004/8/3/medline PY - 2004/7/13/entrez SP - 242 EP - 5 JF - Science (New York, N.Y.) JO - Science VL - 305 IS - 5681 N2 - Numerous degenerative disorders are associated with elevated levels of prooxidants and declines in mitochondrial aconitase activity. Deficiency in the mitochondrial iron-binding protein frataxin results in diminished activity of various mitochondrial iron-sulfur proteins including aconitase. We found that aconitase can undergo reversible citrate-dependent modulation in activity in response to pro-oxidants. Frataxin interacted with aconitase in a citrate-dependent fashion, reduced the level of oxidant-induced inactivation, and converted inactive [3Fe-4S]1+ enzyme to the active [4Fe-4S]2+ form of the protein. Thus, frataxin is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation. SN - 1095-9203 UR - https://www.unboundmedicine.com/medline/citation/15247478/Frataxin_acts_as_an_iron_chaperone_protein_to_modulate_mitochondrial_aconitase_activity_ L2 - https:///www.science.org/doi/10.1126/science.1098991?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -