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Select item 58423761.

ISCU(M108I) and ISCU(D39V) Differ from Wild-Type ISCU in Their Failure To Form Cysteine Desulfurase Complexes Containing Both Frataxin and Ferredoxin

Kai Cai, Ronnie O. Frederick, Marco Tonelli, John L. Markley

Biochemistry. 2018 Mar 6; 57(9): 1491–1500. Published online 2018 Feb 6. doi: 10.1021/acs.biochem.7b01234

PMCID:: PMC5842376

Abstract Article PDF–3.2M

Select item 46754612.

The Human Iron–Sulfur Assembly Complex Catalyzes the Synthesis of [2Fe-2S] Clusters on ISCU2 That Can Be Transferred to Acceptor Molecules

Nicholas G. Fox, Mrinmoy Chakrabarti, Sean P. McCormick, Paul A. Lindahl, David P. Barondeau

Biochemistry. Author manuscript; available in PMC 2015 Dec 10.

Published in final edited form as: Biochemistry. 2015 Jun 30; 54(25): 3871–3879. Published online 2015 Jun 12. doi: 10.1021/bi5014485

PMCID:: PMC4675461

Abstract Article PDF–645K

Select item 35047263.

Tissue Specificity of a Human Mitochondrial Disease: DIFFERENTIATION-ENHANCED MIS-SPLICING OF THE Fe-S SCAFFOLD GENE ISCU RENDERS PATIENT CELLS MORE SENSITIVE TO OXIDATIVE STRESS IN ISCU MYOPATHY

Daniel R. Crooks, Suh Young Jeong, Wing-Hang Tong, Manik C. Ghosh, Hayden Olivierre, Ronald G. Haller, Tracey A. Rouault

J Biol Chem. 2012 Nov 23; 287(48): 40119–40130. Published online 2012 Oct 3. doi: 10.1074/jbc.M112.418889

PMCID:: PMC3504726

Abstract Article PDF–3.4M

Select item 24272124.

Splice Mutation in the Iron-Sulfur Cluster Scaffold Protein ISCU Causes Myopathy with Exercise Intolerance

Fanny Mochel, Melanie A. Knight, Wing-Hang Tong, Dena Hernandez, Karen Ayyad, Tanja Taivassalo, Peter M. Andersen, Andrew Singleton, Tracey A. Rouault, Kenneth H. Fischbeck, Ronald G. Haller

Am J Hum Genet. 2008 Mar 3; 82(3): 652–660. Published online 2008 Feb 29. doi: 10.1016/j.ajhg.2007.12.012

PMCID:: PMC2427212

Abstract Article PDF–1.8M

Select item 60982465.

Zinc(II) binding on human wild-type ISCU and Met140 variants modulates NFS1 desulfurase activity

Nicholas G. Fox, Alain Martelli, Joseph F. Nabhan, Jay Janz, Oktawia Borkowska, Christine Bulawa, Wyatt W. Yue

Biochimie. 2018 Sep; 152: 211–218. doi: 10.1016/j.biochi.2018.07.012

PMCID:: PMC6098246

Abstract Article PDF–967K

Select item 60786416.

Use of antisense oligonucleotides to correct the splicing error in ISCU myopathy patient cell lines

Gregory P. Holmes-Hampton, Daniel R. Crooks, Ronald G. Haller, Shuling Guo, Susan M. Freier, Brett P. Monia, Tracey A. Rouault

Hum Mol Genet. 2016 Dec 1; 25(23): 5178–5187. Published online 2016 Oct 7. doi: 10.1093/hmg/ddw338

PMCID:: PMC6078641

Abstract Article PDF–509K

Select item 31600827.

miR-210 Targets Iron-Sulfur Cluster Scaffold Homologue in Human Trophoblast Cell Lines: Siderosis of Interstitial Trophoblasts as a Novel Pathology of Preterm Preeclampsia and Small-for-Gestational-Age Pregnancies

Deug-Chan Lee, Roberto Romero, Jung-Sun Kim, Adi L. Tarca, Daniel Montenegro, Beth L. Pineles, Ernest Kim, JoonHo Lee, Sun Young Kim, Sorin Draghici, Pooja Mittal, Juan Pedro Kusanovic, Tinnakorn Chaiworapongsa, Sonia S. Hassan, Chong Jai Kim

Am J Pathol. 2011 Aug; 179(2): 590–602. doi: 10.1016/j.ajpath.2011.04.035

PMCID:: PMC3160082

Abstract Article PDF–2.9M

Select item 86171938.

N-terminal tyrosine of ISCU2 triggers [2Fe-2S] cluster synthesis by ISCU2 dimerization

Sven-A. Freibert, Michal T. Boniecki, Claudia Stümpfig, Vinzent Schulz, Nils Krapoth, Dennis R. Winge, Ulrich Mühlenhoff, Oliver Stehling, Miroslaw Cygler, Roland Lill

Nat Commun. 2021; 12: 6902. Published online 2021 Nov 25. doi: 10.1038/s41467-021-27122-w

PMCID:: PMC8617193

Abstract Article PDF–8.6M

Select item 79152019.

Characterization and Reconstitution of Human Lipoyl Synthase (LIAS) Supports ISCA2 and ISCU as Primary Cluster Donors and an Ordered Mechanism of Cluster Assembly

Amber L. Hendricks, Christine Wachnowsky, Brian Fries, Insiya Fidai, James A. Cowan

Int J Mol Sci. 2021 Feb; 22(4): 1598. Published online 2021 Feb 5. doi: 10.3390/ijms22041598

PMCID:: PMC7915201

Abstract Article PDF–1.2M

Select item 607558810.

Cytosolic HSC20 integrates de novo iron–sulfur cluster biogenesis with the CIAO1-mediated transfer to recipients

Ki Soon Kim, Nunziata Maio, Anamika Singh, Tracey A Rouault

Hum Mol Genet. 2018 Mar 1; 27(5): 837–852. Published online 2018 Jan 3. doi: 10.1093/hmg/ddy004

PMCID:: PMC6075588

Abstract Article PDF–1.3M

Select item 574055511.

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

Andrea Legati, Aurelio Reyes, Camilla Ceccatelli Berti, Oliver Stehling, Silvia Marchet, Costanza Lamperti, Alberto Ferrari, Alan J Robinson, Ulrich Mühlenhoff, Roland Lill, Massimo Zeviani, Paola Goffrini, Daniele Ghezzi

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

PMCID:: PMC5740555

Abstract Article PDF–2.1M

Select item 421590112.

Human Frataxin Activates Fe–S Cluster Biosynthesis by Facilitating Sulfur Transfer Chemistry

Jennifer Bridwell-Rabb, Nicholas G. Fox, Chi-Lin Tsai, Andrew M. Winn, David P. Barondeau

Biochemistry. 2014 Aug 5; 53(30): 4904–4913. Published online 2014 Jun 27. doi: 10.1021/bi500532e

PMCID:: PMC4215901

Abstract Article PDF–5.0M

Select item 285994613.

MicroRNA-210 Regulates Mitochondrial Free Radical Response to Hypoxia and Krebs Cycle in Cancer Cells by Targeting Iron Sulfur Cluster Protein ISCU

Elena Favaro, Anassuya Ramachandran, Robert McCormick, Harriet Gee, Christine Blancher, Meredith Crosby, Cecilia Devlin, Christopher Blick, Francesca Buffa, Ji-Liang Li, Borivoj Vojnovic, Ricardo Pires das Neves, Peter Glazer, Francisco Iborra, Mircea Ivan, Jiannis Ragoussis, Adrian L. Harris

PLoS One. 2010; 5(4): e10345. Published online 2010 Apr 26. doi: 10.1371/journal.pone.0010345

PMCID:: PMC2859946

Abstract Article PDF–2.2M

Select item 652520514.

Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism

Nicholas G. Fox, Xiaodi Yu, Xidong Feng, Henry J. Bailey, Alain Martelli, Joseph F. Nabhan, Claire Strain-Damerell, Christine Bulawa, Wyatt W. Yue, Seungil Han

Nat Commun. 2019; 10: 2210. Published online 2019 May 17. doi: 10.1038/s41467-019-09989-y

PMCID:: PMC6525205

Abstract Article PDF–2.2M

Select item 464235015.

Regulation of iron homeostasis by the p53-ISCU pathway

Yuki Funauchi, Chizu Tanikawa, Paulisally Hau Yi Lo, Jinichi Mori, Yataro Daigo, Atsushi Takano, Yohei Miyagi, Atsushi Okawa, Yusuke Nakamura, Koichi Matsuda

Sci Rep. 2015; 5: 16497. Published online 2015 Nov 12. doi: 10.1038/srep16497

PMCID:: PMC4642350

Abstract Article PDF–1.8M

Select item 445981316.

Genetic and hypoxic alterations of the microRNA-210-ISCU1/2 axis promote iron–sulfur deficiency and pulmonary hypertension

Kevin White, Yu Lu, Sofia Annis, Andrew E Hale, B Nelson Chau, James E Dahlman, Craig Hemann, Alexander R Opotowsky, Sara O Vargas, Ivan Rosas, Mark A Perrella, Juan C Osorio, Kathleen J Haley, Brian B Graham, Rahul Kumar, Rajan Saggar, Rajeev Saggar, W Dean Wallace, David J Ross, Omar F Khan, Andrew Bader, Bernadette R Gochuico, Majed Matar, Kevin Polach, Nicolai M Johannessen, Haydn M Prosser, Daniel G Anderson, Robert Langer, Jay L Zweier, Laurence A Bindoff, David Systrom, Aaron B Waxman, Richard C Jin, Stephen Y Chan

EMBO Mol Med. 2015 Jun; 7(6): 695–713. Published online 2015 Mar 30. doi: 10.15252/emmm.201404511

PMCID:: PMC4459813

Abstract Article PDF–1.8M

Select item 364233317.

Mammalian Target of Rapamycin Complex 1 (mTORC1)-mediated Phosphorylation Stabilizes ISCU Protein: IMPLICATIONS FOR IRON METABOLISM

Ping La, Guang Yang, Phyllis A. Dennery

J Biol Chem. 2013 May 3; 288(18): 12901–12909. Published online 2013 Mar 18. doi: 10.1074/jbc.M112.424499

PMCID:: PMC3642333

Abstract Article PDF–1.3M

Select item 293585918.

Characterization of the human HSC20, an unusual DnaJ type III protein, involved in iron–sulfur cluster biogenesis

Helge Uhrigshardt, Anamika Singh, Gennadiy Kovtunovych, Manik Ghosh, Tracey A. Rouault

Hum Mol Genet. 2010 Oct 1; 19(19): 3816–3834. Published online 2010 Jul 28. doi: 10.1093/hmg/ddq301

PMCID:: PMC2935859

Abstract Article PDF–785K

Select item 474943919.

Disease-causing SDHAF1 mutations impair transfer of Fe-S clusters to SDHB

Nunziata Maio, Daniele Ghezzi, Daniela Verrigni, Teresa Rizza, Enrico Bertini, Diego Martinelli, Massimo Zeviani, Anamika Singh, Rosalba Carrozzo, Tracey A. Rouault

Cell Metab. Author manuscript; available in PMC 2017 Feb 9.

Published in final edited form as: Cell Metab. 2016 Feb 9; 23(2): 292–302. Published online 2015 Dec 31. doi: 10.1016/j.cmet.2015.12.005

PMCID:: PMC4749439

Abstract Article PDF–2.7M

Select item 378997220.

Human Mitochondrial Chaperone (mtHSP70) and Cysteine Desulfurase (NFS1) Bind Preferentially to the Disordered Conformation, Whereas Co-chaperone (HSC20) Binds to the Structured Conformation of the Iron-Sulfur Cluster Scaffold Protein (ISCU)

Kai Cai, Ronnie O. Frederick, Jin Hae Kim, Nichole M. Reinen, Marco Tonelli, John L. Markley

J Biol Chem. 2013 Oct 4; 288(40): 28755–28770. Published online 2013 Aug 12. doi: 10.1074/jbc.M113.482042

PMCID:: PMC3789972

Abstract Article PDF–4.6M

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