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Depletion of thiol reducing capacity impairs cytosolic but not mitochondrial iron-sulfur protein assembly machineries.

Braymer JJ, Stümpfig M, Thelen S, Mühlenhoff U, Lill R.

Biochim Biophys Acta Mol Cell Res. 2019 Feb;1866(2):240-251. doi: 10.1016/j.bbamcr.2018.11.003. Epub 2018 Nov 10.


Conserved functions of Arabidopsis mitochondrial late-acting maturation factors in the trafficking of iron‑sulfur clusters.

Uzarska MA, Przybyla-Toscano J, Spantgar F, Zannini F, Lill R, Mühlenhoff U, Rouhier N.

Biochim Biophys Acta Mol Cell Res. 2018 Sep;1865(9):1250-1259. doi: 10.1016/j.bbamcr.2018.06.003. Epub 2018 Jun 12.


Fe-S cluster coordination of the chromokinesin KIF4A alters its subcellular localization during mitosis.

Ben-Shimon L, Paul VD, David-Kadoch G, Volpe M, Stümpfig M, Bill E, Mühlenhoff U, Lill R, Ben-Aroya S.

J Cell Sci. 2018 Jun 27;131(12). pii: jcs211433. doi: 10.1242/jcs.211433.


Biochemical Reconstitution and Spectroscopic Analysis of Iron-Sulfur Proteins.

Freibert SA, Weiler BD, Bill E, Pierik AJ, Mühlenhoff U, Lill R.

Methods Enzymol. 2018;599:197-226. doi: 10.1016/bs.mie.2017.11.034. Epub 2018 Jan 10.


The heme synthesis defect of mutants impaired in mitochondrial iron-sulfur protein biogenesis is caused by reversible inhibition of ferrochelatase.

Lange H, Mühlenhoff U, Denzel M, Kispal G, Lill R.

J Biol Chem. 2017 Dec 15;292(50):20656. doi: 10.1074/jbc.AAC117.000997. No abstract available.


Structure and functional dynamics of the mitochondrial Fe/S cluster synthesis complex.

Boniecki MT, Freibert SA, Mühlenhoff U, Lill R, Cygler M.

Nat Commun. 2017 Nov 3;8(1):1287. doi: 10.1038/s41467-017-01497-1.


A novel de novo dominant mutation in ISCU associated with mitochondrial myopathy.

Legati A, Reyes A, Ceccatelli Berti C, Stehling O, Marchet S, Lamperti C, Ferrari A, Robinson AJ, Mühlenhoff U, Lill R, Zeviani M, Goffrini P, Ghezzi D.

J Med Genet. 2017 Dec;54(12):815-824. doi: 10.1136/jmedgenet-2017-104822. Epub 2017 Oct 27.


Specialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fe-4S] proteins.

Mühlenhoff U, Richter N, Pines O, Pierik AJ, Lill R.

J Biol Chem. 2017 Oct 27;292(43):17979. doi: 10.1074/jbc.AAC117.000255. No abstract available.


Mitochondrial Bol1 and Bol3 function as assembly factors for specific iron-sulfur proteins.

Uzarska MA, Nasta V, Weiler BD, Spantgar F, Ciofi-Baffoni S, Saviello MR, Gonnelli L, Mühlenhoff U, Banci L, Lill R.

Elife. 2016 Aug 17;5. pii: e16673. doi: 10.7554/eLife.16673.


Defects in Mitochondrial Iron-Sulfur Cluster Assembly Induce Cysteine S-Polythiolation on Iron-Sulfur Apoproteins.

Christ S, Leichert LI, Willms A, Lill R, Mühlenhoff U.

Antioxid Redox Signal. 2016 Jul 1;25(1):28-40. doi: 10.1089/ars.2015.6599. Epub 2016 Apr 22.


The mitochondrial monothiol glutaredoxin S15 is essential for iron-sulfur protein maturation in Arabidopsis thaliana.

Moseler A, Aller I, Wagner S, Nietzel T, Przybyla-Toscano J, Mühlenhoff U, Lill R, Berndt C, Rouhier N, Schwarzländer M, Meyer AJ.

Proc Natl Acad Sci U S A. 2015 Nov 3;112(44):13735-40. doi: 10.1073/pnas.1510835112. Epub 2015 Oct 19.


The deca-GX3 proteins Yae1-Lto1 function as adaptors recruiting the ABC protein Rli1 for iron-sulfur cluster insertion.

Paul VD, Mühlenhoff U, Stümpfig M, Seebacher J, Kugler KG, Renicke C, Taxis C, Gavin AC, Pierik AJ, Lill R.

Elife. 2015 Jul 16;4:e08231. doi: 10.7554/eLife.08231.


Compartmentalization of iron between mitochondria and the cytosol and its regulation.

Mühlenhoff U, Hoffmann B, Richter N, Rietzschel N, Spantgar F, Stehling O, Uzarska MA, Lill R.

Eur J Cell Biol. 2015 Jul-Sep;94(7-9):292-308. doi: 10.1016/j.ejcb.2015.05.003. Epub 2015 May 30. Review.


The role of mitochondria and the CIA machinery in the maturation of cytosolic and nuclear iron-sulfur proteins.

Lill R, Dutkiewicz R, Freibert SA, Heidenreich T, Mascarenhas J, Netz DJ, Paul VD, Pierik AJ, Richter N, Stümpfig M, Srinivasan V, Stehling O, Mühlenhoff U.

Eur J Cell Biol. 2015 Jul-Sep;94(7-9):280-91. doi: 10.1016/j.ejcb.2015.05.002. Epub 2015 May 31. Review.


The role of mitochondria in cytosolic-nuclear iron–sulfur protein biogenesis and in cellular iron regulation.

Lill R, Srinivasan V, Mühlenhoff U.

Curr Opin Microbiol. 2014 Dec;22:111-9. doi: 10.1016/j.mib.2014.09.015. Review.


The basic leucine zipper stress response regulator Yap5 senses high-iron conditions by coordination of [2Fe-2S] clusters.

Rietzschel N, Pierik AJ, Bill E, Lill R, Mühlenhoff U.

Mol Cell Biol. 2015 Jan;35(2):370-8. doi: 10.1128/MCB.01033-14. Epub 2014 Nov 3.


Functional reconstitution of mitochondrial Fe/S cluster synthesis on Isu1 reveals the involvement of ferredoxin.

Webert H, Freibert SA, Gallo A, Heidenreich T, Linne U, Amlacher S, Hurt E, Mühlenhoff U, Banci L, Lill R.

Nat Commun. 2014 Oct 31;5:5013. doi: 10.1038/ncomms6013.


The Janus transcription factor HapX controls fungal adaptation to both iron starvation and iron excess.

Gsaller F, Hortschansky P, Beattie SR, Klammer V, Tuppatsch K, Lechner BE, Rietzschel N, Werner ER, Vogan AA, Chung D, Mühlenhoff U, Kato M, Cramer RA, Brakhage AA, Haas H.

EMBO J. 2014 Oct 1;33(19):2261-76. doi: 10.15252/embj.201489468. Epub 2014 Aug 4.


The mitochondrial carrier Rim2 co-imports pyrimidine nucleotides and iron.

Froschauer EM, Rietzschel N, Hassler MR, Binder M, Schweyen RJ, Lill R, Mühlenhoff U, Wiesenberger G.

Biochem J. 2013 Oct 1;455(1):57-65. doi: 10.1042/BJ20130144.


Crucial function of vertebrate glutaredoxin 3 (PICOT) in iron homeostasis and hemoglobin maturation.

Haunhorst P, Hanschmann EM, Bräutigam L, Stehling O, Hoffmann B, Mühlenhoff U, Lill R, Berndt C, Lillig CH.

Mol Biol Cell. 2013 Jun;24(12):1895-903. doi: 10.1091/mbc.E12-09-0648. Epub 2013 Apr 24.


The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation.

Uzarska MA, Dutkiewicz R, Freibert SA, Lill R, Mühlenhoff U.

Mol Biol Cell. 2013 Jun;24(12):1830-41. doi: 10.1091/mbc.E12-09-0644. Epub 2013 Apr 24.


The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism.

Lill R, Hoffmann B, Molik S, Pierik AJ, Rietzschel N, Stehling O, Uzarska MA, Webert H, Wilbrecht C, Mühlenhoff U.

Biochim Biophys Acta. 2012 Sep;1823(9):1491-508. doi: 10.1016/j.bbamcr.2012.05.009. Epub 2012 May 15. Review.


The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation.

Sheftel AD, Wilbrecht C, Stehling O, Niggemeyer B, Elsässer HP, Mühlenhoff U, Lill R.

Mol Biol Cell. 2012 Apr;23(7):1157-66. doi: 10.1091/mbc.E11-09-0772. Epub 2012 Feb 9.


Specialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fe-4S] proteins.

Mühlenhoff U, Richter N, Pines O, Pierik AJ, Lill R.

J Biol Chem. 2011 Dec 2;286(48):41205-16. doi: 10.1074/jbc.M111.296152. Epub 2011 Oct 10. Erratum in: J Biol Chem. 2017 Oct 27;292(43):17979.


Coenzyme Q biosynthesis: Coq6 is required for the C5-hydroxylation reaction and substrate analogs rescue Coq6 deficiency.

Ozeir M, Mühlenhoff U, Webert H, Lill R, Fontecave M, Pierrel F.

Chem Biol. 2011 Sep 23;18(9):1134-42. doi: 10.1016/j.chembiol.2011.07.008.


The oxidative stress response in yeast cells involves changes in the stability of Aft1 regulon mRNAs.

Castells-Roca L, Mühlenhoff U, Lill R, Herrero E, Bellí G.

Mol Microbiol. 2011 Jul;81(1):232-48. doi: 10.1111/j.1365-2958.2011.07689.x. Epub 2011 May 25.


The multidomain thioredoxin-monothiol glutaredoxins represent a distinct functional group.

Hoffmann B, Uzarska MA, Berndt C, Godoy JR, Haunhorst P, Lillig CH, Lill R, Mühlenhoff U.

Antioxid Redox Signal. 2011 Jul 1;15(1):19-30. doi: 10.1089/ars.2010.3811. Epub 2011 Apr 20.


Cytosolic monothiol glutaredoxins function in intracellular iron sensing and trafficking via their bound iron-sulfur cluster.

Mühlenhoff U, Molik S, Godoy JR, Uzarska MA, Richter N, Seubert A, Zhang Y, Stubbe J, Pierrel F, Herrero E, Lillig CH, Lill R.

Cell Metab. 2010 Oct 6;12(4):373-385. doi: 10.1016/j.cmet.2010.08.001. Erratum in: Cell Metab. 2014 Oct 7;20(4):696.


Tah18 transfers electrons to Dre2 in cytosolic iron-sulfur protein biogenesis.

Netz DJ, Stümpfig M, Doré C, Mühlenhoff U, Pierik AJ, Lill R.

Nat Chem Biol. 2010 Oct;6(10):758-65. doi: 10.1038/nchembio.432. Epub 2010 Aug 29.


Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis.

Sheftel AD, Stehling O, Pierik AJ, Elsässer HP, Mühlenhoff U, Webert H, Hobler A, Hannemann F, Bernhardt R, Lill R.

Proc Natl Acad Sci U S A. 2010 Jun 29;107(26):11775-80. doi: 10.1073/pnas.1004250107. Epub 2010 Jun 14.


Involvement of mitochondrial ferredoxin and para-aminobenzoic acid in yeast coenzyme Q biosynthesis.

Pierrel F, Hamelin O, Douki T, Kieffer-Jaquinod S, Mühlenhoff U, Ozeir M, Lill R, Fontecave M.

Chem Biol. 2010 May 28;17(5):449-59. doi: 10.1016/j.chembiol.2010.03.014.


Iron regulation through the back door: iron-dependent metabolite levels contribute to transcriptional adaptation to iron deprivation in Saccharomyces cerevisiae.

Ihrig J, Hausmann A, Hain A, Richter N, Hamza I, Lill R, Mühlenhoff U.

Eukaryot Cell. 2010 Mar;9(3):460-71. doi: 10.1128/EC.00213-09. Epub 2009 Dec 11.


Crucial role of conserved cysteine residues in the assembly of two iron-sulfur clusters on the CIA protein Nar1.

Urzica E, Pierik AJ, Mühlenhoff U, Lill R.

Biochemistry. 2009 Jun 9;48(22):4946-58. doi: 10.1021/bi900312x.


Saccharomyces cerevisiae Grx6 and Grx7 are monothiol glutaredoxins associated with the early secretory pathway.

Izquierdo A, Casas C, Mühlenhoff U, Lillig CH, Herrero E.

Eukaryot Cell. 2008 Aug;7(8):1415-26. doi: 10.1128/EC.00133-08. Epub 2008 May 23.


Maturation of iron-sulfur proteins in eukaryotes: mechanisms, connected processes, and diseases.

Lill R, Mühlenhoff U.

Annu Rev Biochem. 2008;77:669-700. doi: 10.1146/annurev.biochem.76.052705.162653. Review.


Investigation of iron-sulfur protein maturation in eukaryotes.

Stehling O, Smith PM, Biederbick A, Balk J, Lill R, Mühlenhoff U.

Methods Mol Biol. 2007;372:325-42. doi: 10.1007/978-1-59745-365-3_24.


Cellular and mitochondrial remodeling upon defects in iron-sulfur protein biogenesis.

Hausmann A, Samans B, Lill R, Mühlenhoff U.

J Biol Chem. 2008 Mar 28;283(13):8318-30. doi: 10.1074/jbc.M705570200. Epub 2008 Jan 28.


Mitochondrial Iba57p is required for Fe/S cluster formation on aconitase and activation of radical SAM enzymes.

Gelling C, Dawes IW, Richhardt N, Lill R, Mühlenhoff U.

Mol Cell Biol. 2008 Mar;28(5):1851-61. Epub 2007 Dec 17.


Methods for studying iron metabolism in yeast mitochondria.

Molik S, Lill R, Mühlenhoff U.

Methods Cell Biol. 2007;80:261-80. Review. No abstract available.


The Cfd1-Nbp35 complex acts as a scaffold for iron-sulfur protein assembly in the yeast cytosol.

Netz DJ, Pierik AJ, Stümpfig M, Mühlenhoff U, Lill R.

Nat Chem Biol. 2007 May;3(5):278-86. Epub 2007 Apr 1.


The ISC [corrected] proteins Isa1 and Isa2 are required for the function but not for the de novo synthesis of the Fe/S clusters of biotin synthase in Saccharomyces cerevisiae.

Mühlenhoff U, Gerl MJ, Flauger B, Pirner HM, Balser S, Richhardt N, Lill R, Stolz J.

Eukaryot Cell. 2007 Mar;6(3):495-504. Epub 2007 Jan 26. Erratum in: Eukaryot Cell. 2007 Apr;6(4):753.


Application of the DNA-specific dye EvaGreen for the routine quantification of DNA in microplates.

Ihrig J, Lill R, Mühlenhoff U.

Anal Biochem. 2006 Dec 15;359(2):265-7. Epub 2006 Aug 18. No abstract available.


Mechanisms of iron-sulfur protein maturation in mitochondria, cytosol and nucleus of eukaryotes.

Lill R, Dutkiewicz R, Elsässer HP, Hausmann A, Netz DJ, Pierik AJ, Stehling O, Urzica E, Mühlenhoff U.

Biochim Biophys Acta. 2006 Jul;1763(7):652-67. Epub 2006 May 23. Review.


Iron-sulfur protein biogenesis in eukaryotes: components and mechanisms.

Lill R, Mühlenhoff U.

Annu Rev Cell Dev Biol. 2006;22:457-86. Review.


Stimulation of the ATPase activity of the yeast mitochondrial ABC transporter Atm1p by thiol compounds.

Kuhnke G, Neumann K, Mühlenhoff U, Lill R.

Mol Membr Biol. 2006 Mar-Apr;23(2):173-84.


Role of glutaredoxin-3 and glutaredoxin-4 in the iron regulation of the Aft1 transcriptional activator in Saccharomyces cerevisiae.

Ojeda L, Keller G, Muhlenhoff U, Rutherford JC, Lill R, Winge DR.

J Biol Chem. 2006 Jun 30;281(26):17661-9. Epub 2006 Apr 28.


The Hsp70 chaperone Ssq1p is dispensable for iron-sulfur cluster formation on the scaffold protein Isu1p.

Dutkiewicz R, Marszalek J, Schilke B, Craig EA, Lill R, Mühlenhoff U.

J Biol Chem. 2006 Mar 24;281(12):7801-8. Epub 2006 Jan 23.


Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins.

Wiedemann N, Urzica E, Guiard B, Müller H, Lohaus C, Meyer HE, Ryan MT, Meisinger C, Mühlenhoff U, Lill R, Pfanner N.

EMBO J. 2006 Jan 11;25(1):184-95. Epub 2005 Dec 8.


Iron-sulfur-protein biogenesis in eukaryotes.

Lill R, Mühlenhoff U.

Trends Biochem Sci. 2005 Mar;30(3):133-41. Review.


The eukaryotic P loop NTPase Nbp35: an essential component of the cytosolic and nuclear iron-sulfur protein assembly machinery.

Hausmann A, Aguilar Netz DJ, Balk J, Pierik AJ, Mühlenhoff U, Lill R.

Proc Natl Acad Sci U S A. 2005 Mar 1;102(9):3266-71. Epub 2005 Feb 22.

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