{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T16:30:36Z","timestamp":1791563436763,"version":"4.3.4"},"reference-count":83,"publisher":"The Company of Biologists","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,3,1]]},"abstract":"<jats:p>Iron-sulfur (Fe-S) clusters are ubiquitous cofactors composed of iron and inorganic sulfur. They are required for the function of proteins involved in a wide range of activities, including electron transport in respiratory chain complexes, regulatory sensing, photosynthesis and DNA repair. The proteins involved in the biogenesis of Fe-S clusters are evolutionarily conserved from bacteria to humans, and many insights into the process of Fe-S cluster biogenesis have come from studies of model organisms, including bacteria, fungi and plants. It is now clear that several rare and seemingly dissimilar human diseases are attributable to defects in the basic process of Fe-S cluster biogenesis. Although these diseases \u2013which include Friedreich\u2019s ataxia (FRDA), ISCU myopathy, a rare form of sideroblastic anemia, an encephalomyopathy caused by dysfunction of respiratory chain complex I and multiple mitochondrial dysfunctions syndrome \u2013 affect different tissues, a feature common to many of them is that mitochondrial iron overload develops as a secondary consequence of a defect in Fe-S cluster biogenesis. This Commentary outlines the basic steps of Fe-S cluster biogenesis as they have been defined in model organisms. In addition, it draws attention to refinements of the process that might be specific to the subcellular compartmentalization of Fe-S cluster biogenesis proteins in some eukaryotes, including mammals. Finally, it outlines several important unresolved questions in the field that, once addressed, should offer important clues into how mitochondrial iron homeostasis is regulated, and how dysfunction in Fe-S cluster biogenesis can contribute to disease.<\/jats:p>","DOI":"10.1242\/dmm.009019","type":"journal-article","created":{"date-parts":[[2012,3,2]],"date-time":"2012-03-02T15:12:12Z","timestamp":1330701132000},"page":"155-164","source":"Crossref","is-referenced-by-count":314,"title":["Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease"],"prefix":"10.1242","volume":"5","author":[{"given":"Tracey A.","family":"Rouault","sequence":"first","affiliation":[{"name":"National Institute of Child Health and Human Development, Bethesda, MD 20892, USA e-mail: Rouault@mail.nih.gov"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","reference":[{"key":"2021042612511039000_b1-0050155","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1038\/sj.emboj.7600905","article-title":"The Nfs1 interacting protein Isd11 has an essential role in Fe\/S cluster biogenesis in mitochondria","volume":"25","author":"Adam","year":"2006","journal-title":"EMBO J."},{"key":"2021042612511039000_b2-0050155","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1093\/hmg\/8.5.743","article-title":"Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA\/A)","volume":"8","author":"Allikmets","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"2021042612511039000_b3-0050155","doi-asserted-by":"crossref","first-page":"1709","DOI":"10.1126\/science.276.5319.1709","article-title":"Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin","volume":"276","author":"Babcock","year":"1997","journal-title":"Science"},{"key":"2021042612511039000_b4-0050155","doi-asserted-by":"crossref","first-page":"1112","DOI":"10.1042\/BST0361112","article-title":"Iron-sulfur cluster biosynthesis","volume":"36","author":"Bandyopadhyay","year":"2008","journal-title":"Biochem. Soc. Trans."},{"key":"2021042612511039000_b5-0050155","doi-asserted-by":"crossref","first-page":"40878","DOI":"10.1074\/jbc.M111.270082","article-title":"Leucine biosynthesis regulates cytoplasmic iron-sulfur enzyme biogenesis in an Atm1-independent manner","volume":"286","author":"Bedekovics","year":"2011","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b6-0050155","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1007\/s007750050002","article-title":"Iron-sulfur proteins: ancient structures, still full of surprises","volume":"5","author":"Beinert","year":"2000","journal-title":"J. Biol. Inorg. Chem."},{"key":"2021042612511039000_b7-0050155","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1126\/science.277.5326.653","article-title":"Iron-sulfur clusters: nature\u2019s modular, multipurpose structures","volume":"277","author":"Beinert","year":"1997","journal-title":"Science"},{"key":"2021042612511039000_b8-0050155","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1002\/pbc.22244","article-title":"Systematic molecular genetic analysis of congenital sideroblastic anemia: evidence for genetic heterogeneity and identification of novel mutations","volume":"54","author":"Bergmann","year":"2010","journal-title":"Pediatr. Blood Cancer"},{"key":"2021042612511039000_b9-0050155","doi-asserted-by":"crossref","first-page":"5675","DOI":"10.1128\/MCB.00112-06","article-title":"Role of human mitochondrial Nfs1 in cytosolic iron-sulfur protein biogenesis and iron regulation","volume":"26","author":"Biederbick","year":"2006","journal-title":"Mol. Cell. Biol."},{"key":"2021042612511039000_b10-0050155","doi-asserted-by":"crossref","first-page":"30184","DOI":"10.1074\/jbc.M804746200","article-title":"Structure of human J-type co-chaperone HscB reveals a tetracysteine metal-binding domain","volume":"283","author":"Bitto","year":"2008","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b11-0050155","doi-asserted-by":"crossref","first-page":"9641","DOI":"10.1021\/bi201123z","article-title":"Facilitated transfer of IscU-[2Fe2S] clusters by chaperone-mediated ligand exchange","volume":"50","author":"Bonomi","year":"2011","journal-title":"Biochemistry"},{"key":"2021042612511039000_b12-0050155","doi-asserted-by":"crossref","first-page":"7265","DOI":"10.1021\/bi200895k","article-title":"Structure-function analysis of Friedreich\u2019s ataxia mutants reveals determinants for frataxin binding and activation of the Fe-S assembly complex","volume":"50","author":"Bridwell-Rabb","year":"2011","journal-title":"Biochemistry"},{"key":"2021042612511039000_b13-0050155","doi-asserted-by":"crossref","first-page":"1736","DOI":"10.1038\/emboj.2008.98","article-title":"The iron-sulphur protein Ind1 is required for effective complex I assembly","volume":"27","author":"Bych","year":"2008","journal-title":"EMBO J."},{"key":"2021042612511039000_b14-0050155","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1038\/ng.659","article-title":"High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency","volume":"42","author":"Calvo","year":"2010","journal-title":"Nat. Genet."},{"key":"2021042612511039000_b15-0050155","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1182\/blood-2007-02-072520","article-title":"The human counterpart of zebrafish shiraz shows sideroblastic-like microcytic anemia and iron overload","volume":"110","author":"Camaschella","year":"2007","journal-title":"Blood"},{"key":"2021042612511039000_b16-0050155","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1016\/j.ajhg.2011.08.011","article-title":"Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes","volume":"89","author":"Cameron","year":"2011","journal-title":"Am. J. Hum. Genet."},{"key":"2021042612511039000_b17-0050155","doi-asserted-by":"crossref","first-page":"6804","DOI":"10.1021\/bi6026659","article-title":"Formation and properties of [4Fe-4S] clusters on the IscU scaffold protein","volume":"46","author":"Chandramouli","year":"2007","journal-title":"Biochemistry"},{"key":"2021042612511039000_b18-0050155","doi-asserted-by":"crossref","first-page":"2035","DOI":"10.1242\/jcs.02922","article-title":"Motor protein KIFC5A interacts with Nubp1 and Nubp2, and is implicated in the regulation of centrosome duplication","volume":"119","author":"Christodoulou","year":"2006","journal-title":"J. Cell Sci."},{"key":"2021042612511039000_b19-0050155","doi-asserted-by":"crossref","first-page":"8756","DOI":"10.1021\/bi1008613","article-title":"Molecular details of the yeast frataxin-Isu1 interaction during mitochondrial Fe-S cluster assembly","volume":"49","author":"Cook","year":"2010","journal-title":"Biochemistry"},{"key":"2021042612511039000_b20-0050155","doi-asserted-by":"crossref","first-page":"1658","DOI":"10.1007\/PL00012493","article-title":"A specialized mitochondrial molecular chaperone system: a role in formation of Fe\/S centers","volume":"59","author":"Craig","year":"2002","journal-title":"Cell. Mol. Life Sci."},{"key":"2021042612511039000_b21-0050155","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.bbapap.2010.06.003","article-title":"Adrenodoxin: the archetype of vertebrate-type [2Fe-2S] cluster ferredoxins","volume":"1814","author":"Ewen","year":"2011","journal-title":"Biochim. Biophys. Acta"},{"key":"2021042612511039000_b22-0050155","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1136\/jmg.2006.045252","article-title":"Nonsense mutation in pseudouridylate synthase 1 (PUS1) in two brothers affected by myopathy, lactic acidosis and sideroblastic anaemia (MLASA)","volume":"44","author":"Fernandez-Vizarra","year":"2007","journal-title":"J. Med. Genet."},{"key":"2021042612511039000_b23-0050155","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/S1367-5931(03)00021-8","article-title":"Formation of iron-sulfur clusters in bacteria: an emerging field in bioinorganic chemistry","volume":"7","author":"Frazzon","year":"2003","journal-title":"Curr. Opin. Chem. Biol."},{"key":"2021042612511039000_b24-0050155","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/1471-2091-12-3","article-title":"Three hydrophobic amino acids in Escherichia coli HscB make the greatest contribution to the stability of the HscB-IscU complex","volume":"12","author":"Fuzery","year":"2011","journal-title":"BMC Biochem."},{"key":"2021042612511039000_b25-0050155","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1038\/ng.359","article-title":"Mutations in mitochondrial carrier family gene SLC25A38 cause nonsyndromic autosomal recessive congenital sideroblastic anemia","volume":"41","author":"Guernsey","year":"2009","journal-title":"Nat. Genet."},{"key":"2021042612511039000_b26-0050155","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1007\/s10534-011-9476-8","article-title":"Glutathione: a key component of the cytoplasmic labile iron pool","volume":"24","author":"Hider","year":"2011","journal-title":"Biometals"},{"key":"2021042612511039000_b27-0050155","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1042\/BJ20091382","article-title":"Towards the molecular mechanism of respiratory complex I","volume":"425","author":"Hirst","year":"2010","journal-title":"Biochem. J."},{"key":"2021042612511039000_b28-0050155","doi-asserted-by":"crossref","first-page":"16381","DOI":"10.1073\/pnas.0906784106","article-title":"Elucidation of the mechanism of mitochondrial iron loading in Friedreich\u2019s ataxia by analysis of a mouse mutant","volume":"106","author":"Huang","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042612511039000_b29-0050155","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1016\/j.febslet.2004.11.111","article-title":"Combining data from genomes, Y2H and 3D structure indicates that BolA is a reductase interacting with a glutaredoxin","volume":"579","author":"Huynen","year":"2005","journal-title":"FEBS Lett."},{"key":"2021042612511039000_b30-0050155","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1111\/j.1365-2958.2006.05028.x","article-title":"Iron-sulphur clusters and the problem with oxygen","volume":"59","author":"Imlay","year":"2006","journal-title":"Mol. Microbiol."},{"key":"2021042612511039000_b31-0050155","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1038\/nrm2941","article-title":"The HSP70 chaperone machinery: J proteins as drivers of functional specificity","volume":"11","author":"Kampinga","year":"2010","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"2021042612511039000_b32-0050155","doi-asserted-by":"crossref","first-page":"808","DOI":"10.3324\/haematol.2010.039164","article-title":"Missense SLC25A38 variations play an important role in autosomal recessive inherited sideroblastic anemia","volume":"96","author":"Kannengiesser","year":"2011","journal-title":"Haematologica"},{"key":"2021042612511039000_b33-0050155","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S1369-5274(03)00039-0","article-title":"The role of Fe-S proteins in sensing and regulation in bacteria","volume":"6","author":"Kiley","year":"2003","journal-title":"Curr. Opin. Microbiol."},{"key":"2021042612511039000_b34-0050155","doi-asserted-by":"crossref","first-page":"17524","DOI":"10.1074\/jbc.M010695200","article-title":"J-domain protein, Jac1p, of yeast mitochondria required for iron homeostasis and activity of Fe-S cluster proteins","volume":"276","author":"Kim","year":"2001","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b35-0050155","doi-asserted-by":"crossref","first-page":"3981","DOI":"10.1093\/emboj\/18.14.3981","article-title":"The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe\/S proteins","volume":"18","author":"Kispal","year":"1999","journal-title":"EMBO J."},{"key":"2021042612511039000_b36-0050155","doi-asserted-by":"crossref","first-page":"18389","DOI":"10.1074\/jbc.273.29.18389","article-title":"Mt-Hsp70 homolog, Ssc2p, required for maturation of yeast frataxin and mitochondrial iron homeostasis","volume":"273","author":"Knight","year":"1998","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b37-0050155","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jns.2011.01.010","article-title":"Friedreich\u2019s ataxia: pathology, pathogenesis, and molecular genetics","volume":"303","author":"Koeppen","year":"2011","journal-title":"J. Neurol. Sci."},{"key":"2021042612511039000_b38-0050155","doi-asserted-by":"crossref","first-page":"2170","DOI":"10.1093\/brain\/awp152","article-title":"Clinical manifestation and a new ISCU mutation in iron-sulphur cluster deficiency myopathy","volume":"132","author":"Kollberg","year":"2009","journal-title":"Brain"},{"key":"2021042612511039000_b39-0050155","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1080\/09687860500473630","article-title":"Stimulation of the ATPase activity of the yeast mitochondrial ABC transporter Atm1p by thiol compounds","volume":"23","author":"Kuhnke","year":"2006","journal-title":"Mol. Membr. Biol."},{"key":"2021042612511039000_b40-0050155","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1016\/S1097-2765(00)80295-6","article-title":"Targeting of a human iron-sulfur cluster assembly enzyme, nifs, to different subcellular compartments is regulated through alternative AUG utilization","volume":"2","author":"Land","year":"1998","journal-title":"Mol. Cell"},{"key":"2021042612511039000_b41-0050155","doi-asserted-by":"crossref","first-page":"2265","DOI":"10.1093\/hmg\/ddn127","article-title":"Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia","volume":"17","author":"Li","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"2021042612511039000_b42-0050155","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1038\/nature08301","article-title":"Function and biogenesis of iron-sulphur proteins","volume":"460","author":"Lill","year":"2009","journal-title":"Nature"},{"key":"2021042612511039000_b43-0050155","doi-asserted-by":"crossref","first-page":"1304","DOI":"10.1016\/j.bbagen.2008.06.003","article-title":"Glutaredoxin systems","volume":"1780","author":"Lillig","year":"2008","journal-title":"Biochim. Biophys. Acta"},{"key":"2021042612511039000_b44-0050155","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1242\/dmm.008706","article-title":"Understanding the genetic and molecular pathogenesis of Friedreich\u2019s ataxia through animal and cellular models","volume":"5","author":"Martelli","year":"2012","journal-title":"Dis. Model. Mech."},{"key":"2021042612511039000_b45-0050155","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nrmicro1991","article-title":"Hydrothermal vents and the origin of life","volume":"6","author":"Martin","year":"2008","journal-title":"Nat. Rev. Microbiol."},{"key":"2021042612511039000_b46-0050155","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s00775-007-0318-7","article-title":"Iron-sulfur protein folds, iron-sulfur chemistry, and evolution","volume":"13","author":"Meyer","year":"2008","journal-title":"J. Biol. Inorg. Chem."},{"key":"2021042612511039000_b47-0050155","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1016\/j.ajhg.2007.12.012","article-title":"Splice mutation in the iron-sulfur cluster scaffold protein ISCU causes myopathy with exercise intolerance","volume":"82","author":"Mochel","year":"2008","journal-title":"Am. J. Hum. Genet."},{"key":"2021042612511039000_b48-0050155","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1146\/annurev.nutr.28.061807.155521","article-title":"Systemic iron homeostasis and the iron-responsive element\/iron-regulatory protein (IRE\/IRP) regulatory network","volume":"28","author":"Muckenthaler","year":"2008","journal-title":"Annu. Rev. Nutr."},{"key":"2021042612511039000_b49-0050155","doi-asserted-by":"crossref","first-page":"8314","DOI":"10.1074\/jbc.M007878200","article-title":"Nuclear localization of yeast Nfs1p is required for cell survival","volume":"276","author":"Nakai","year":"2001","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b50-0050155","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1016\/j.ajhg.2011.10.005","article-title":"A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins","volume":"89","author":"Navarro-Sastre","year":"2011","journal-title":"Am. J. Hum. Genet."},{"key":"2021042612511039000_b51-0050155","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1038\/nchembio.432","article-title":"Tah18 transfers electrons to Dre2 in cytosolic iron-sulfur protein biogenesis","volume":"6","author":"Netz","year":"2010","journal-title":"Nat. Chem. Biol."},{"key":"2021042612511039000_b52-0050155","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1038\/nchembio.721","article-title":"Eukaryotic DNA polymerases require an iron-sulfur cluster for the formation of active complexes","volume":"8","author":"Netz","year":"2011","journal-title":"Nat. Chem. Biol."},{"key":"2021042612511039000_b53-0050155","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22002","article-title":"The defective splicing caused by the ISCU intron mutation in patients with myopathy with lactic acidosis is repressed by PTBP1 but can be de-repressed by IGF2BP1","author":"Nordin","year":"2011","journal-title":"Hum. Mutat."},{"key":"2021042612511039000_b54-0050155","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1093\/hmg\/ddn057","article-title":"Myopathy with lactic acidosis is linked to chromosome 12q23.3-24.11 and caused by an intron mutation in the ISCU gene resulting in a splicing defect","volume":"17","author":"Olsson","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"2021042612511039000_b55-0050155","doi-asserted-by":"crossref","first-page":"3567","DOI":"10.1182\/blood-2006-04-015768","article-title":"Abcb7, the gene responsible for X-linked sideroblastic anemia with ataxia, is essential for hematopoiesis","volume":"109","author":"Pondarre","year":"2007","journal-title":"Blood"},{"key":"2021042612511039000_b56-0050155","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1038\/ncomms1097","article-title":"Structural bases for the interaction of frataxin with the central components of iron-sulphur cluster assembly","volume":"1","author":"Prischi","year":"2010","journal-title":"Nat. Commun."},{"key":"2021042612511039000_b57-0050155","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1038\/nrmicro2356","article-title":"Building Fe-S proteins: bacterial strategies","volume":"8","author":"Py","year":"2010","journal-title":"Nat. Rev. Microbiol."},{"key":"2021042612511039000_b58-0050155","doi-asserted-by":"crossref","first-page":"4989","DOI":"10.1039\/c0cc05079b","article-title":"Mechanism of glutaredoxin-ISU [2Fe-2S] cluster exchange","volume":"47","author":"Qi","year":"2011","journal-title":"Chem. Commun."},{"key":"2021042612511039000_b59-0050155","doi-asserted-by":"crossref","first-page":"8591","DOI":"10.1073\/pnas.0803173105","article-title":"In vivo iron-sulfur cluster formation","volume":"105","author":"Raulfs","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042612511039000_b60-0050155","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1093\/molbev\/msl001","article-title":"Evolution of the Isd11-IscS complex reveals a single alpha-proteobacterial endosymbiosis for all eukaryotes","volume":"23","author":"Richards","year":"2006","journal-title":"Mol. Biol. Evol."},{"key":"2021042612511039000_b61-0050155","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.ajhg.2010.06.001","article-title":"Mutation of the mitochondrial tyrosyl-tRNA synthetase gene, YARS2, causes myopathy, lactic acidosis, and sideroblastic anemia-MLASA syndrome","volume":"87","author":"Riley","year":"2010","journal-title":"Am. J. Hum. Genet."},{"key":"2021042612511039000_b62-0050155","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1038\/nchembio807","article-title":"The role of iron regulatory proteins in mammalian iron homeostasis and disease","volume":"2","author":"Rouault","year":"2006","journal-title":"Nat. Chem. Biol."},{"key":"2021042612511039000_b63-0050155","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1016\/j.molcel.2006.07.019","article-title":"The DNA repair helicases XPD and FancJ have essential iron-sulfur domains","volume":"23","author":"Rudolf","year":"2006","journal-title":"Mol. Cell"},{"key":"2021042612511039000_b64-0050155","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1016\/j.tibs.2004.05.007","article-title":"The rocky roots of the acetyl-CoA pathway","volume":"29","author":"Russell","year":"2004","journal-title":"Trends Biochem. Sci."},{"key":"2021042612511039000_b65-0050155","doi-asserted-by":"crossref","first-page":"e16199","DOI":"10.1371\/journal.pone.0016199","article-title":"Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU\/NFS1\/ISD11 iron-sulfur assembly complex","volume":"6","author":"Schmucker","year":"2011","journal-title":"PLoS ONE"},{"key":"2021042612511039000_b66-0050155","doi-asserted-by":"crossref","first-page":"26745","DOI":"10.1074\/jbc.R110.122218","article-title":"Cytosolic iron-sulfur cluster assembly (CIA) system: factors, mechanism, and relevance to cellular iron regulation","volume":"285","author":"Sharma","year":"2010","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b67-0050155","doi-asserted-by":"crossref","first-page":"6059","DOI":"10.1128\/MCB.00817-09","article-title":"Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I","volume":"29","author":"Sheftel","year":"2009","journal-title":"Mol. Cell. Biol."},{"key":"2021042612511039000_b68-0050155","doi-asserted-by":"crossref","first-page":"e1000354","DOI":"10.1371\/journal.pbio.1000354","article-title":"Structural basis for Fe-S cluster assembly and tRNA thiolation mediated by IscS protein-protein interactions","volume":"8","author":"Shi","year":"2010","journal-title":"PLoS Biol."},{"key":"2021042612511039000_b69-0050155","doi-asserted-by":"crossref","first-page":"3014","DOI":"10.1093\/hmg\/ddp239","article-title":"Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis","volume":"18","author":"Shi","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"2021042612511039000_b70-0050155","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/j.bbamcr.2011.11.002","article-title":"Both human ferredoxins 1 and 2 and ferredoxin reductase are important for iron-sulfur cluster biogenesis","volume":"1823","author":"Shi","year":"2011","journal-title":"Biochim. Biophys. Acta"},{"key":"2021042612511039000_b71-0050155","doi-asserted-by":"crossref","first-page":"1246","DOI":"10.1016\/j.str.2007.08.009","article-title":"Structure of the yeast WD40 domain protein Cia1, a component acting late in iron-sulfur protein biogenesis","volume":"15","author":"Srinivasan","year":"2007","journal-title":"Structure"},{"key":"2021042612511039000_b72-0050155","doi-asserted-by":"crossref","first-page":"5517","DOI":"10.1128\/MCB.00545-08","article-title":"Human Nbp35 is essential for both cytosolic iron-sulfur protein assembly and iron homeostasis","volume":"28","author":"Stehling","year":"2008","journal-title":"Mol. Cell. Biol."},{"key":"2021042612511039000_b73-0050155","doi-asserted-by":"crossref","first-page":"26737","DOI":"10.1074\/jbc.R110.118679","article-title":"Frataxin and mitochondrial FeS cluster biogenesis","volume":"285","author":"Stemmler","year":"2010","journal-title":"J. Biol. Chem."},{"key":"2021042612511039000_b74-0050155","doi-asserted-by":"crossref","first-page":"5692","DOI":"10.1093\/emboj\/19.21.5692","article-title":"Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells","volume":"19","author":"Tong","year":"2000","journal-title":"EMBO J."},{"key":"2021042612511039000_b75-0050155","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/j.cmet.2006.02.003","article-title":"Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis","volume":"3","author":"Tong","year":"2006","journal-title":"Cell Metab."},{"key":"2021042612511039000_b76-0050155","doi-asserted-by":"crossref","first-page":"9762","DOI":"10.1073\/pnas.1732541100","article-title":"Subcellular compartmentalization of human Nfu, an iron-sulfur cluster scaffold protein, and its ability to assemble a [4Fe-4S] cluster","volume":"100","author":"Tong","year":"2003","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042612511039000_b77-0050155","doi-asserted-by":"crossref","first-page":"9132","DOI":"10.1021\/bi1013062","article-title":"Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex","volume":"49","author":"Tsai","year":"2010","journal-title":"Biochemistry"},{"key":"2021042612511039000_b78-0050155","doi-asserted-by":"crossref","first-page":"3816","DOI":"10.1093\/hmg\/ddq301","article-title":"Characterization of the human HSC20, an unusual DnaJ type III protein, involved in iron-sulfur cluster biogenesis","volume":"19","author":"Uhrigshardt","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"2021042612511039000_b79-0050155","doi-asserted-by":"crossref","first-page":"1647","DOI":"10.1093\/nar\/gkq1045","article-title":"RTEL1: an essential helicase for telomere maintenance and the regulation of homologous recombination","volume":"39","author":"Uringa","year":"2011","journal-title":"Nucleic Acids Res."},{"key":"2021042612511039000_b80-0050155","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1080\/10409230701322298","article-title":"Molecular chaperones HscA\/Ssq1 and HscB\/Jac1 and their roles in iron-sulfur protein maturation","volume":"42","author":"Vickery","year":"2007","journal-title":"Crit. Rev. Biochem. Mol. Biol."},{"key":"2021042612511039000_b81-0050155","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1038\/sj.emboj.7600906","article-title":"Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins","volume":"25","author":"Wiedemann","year":"2006","journal-title":"EMBO J."},{"key":"2021042612511039000_b82-0050155","doi-asserted-by":"crossref","first-page":"4945","DOI":"10.1021\/bi1004798","article-title":"Human iron-sulfur cluster assembly, cellular iron homeostasis, and disease","volume":"49","author":"Ye","year":"2010","journal-title":"Biochemistry"},{"key":"2021042612511039000_b83-0050155","doi-asserted-by":"crossref","first-page":"1749","DOI":"10.1172\/JCI40372","article-title":"Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts","volume":"120","author":"Ye","year":"2010","journal-title":"J. Clin. Invest."}],"container-title":["Disease Models &amp; Mechanisms"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/journals.biologists.com\/dmm\/article-pdf\/5\/2\/155\/1553426\/155.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/journals.biologists.com\/dmm\/article-pdf\/5\/2\/155\/1553426\/155.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,26]],"date-time":"2021-04-26T18:47:21Z","timestamp":1619462841000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/dmm\/article\/5\/2\/155\/53553\/Biogenesis-of-iron-sulfur-clusters-in-mammalian"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,3,1]]},"references-count":83,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2012,3,1]]}},"URL":"https:\/\/doi.org\/10.1242\/dmm.009019","relation":{},"ISSN":["1754-8411","1754-8403"],"issn-type":[{"value":"1754-8411","type":"electronic"},{"value":"1754-8403","type":"print"}],"subject":[],"published":{"date-parts":[[2012,3,1]]}}}