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Select item 103881781.

Structural Plasticity of NFU1 Upon Interaction with Binding Partners: Insights into the Mitochondrial [4Fe-4S] Cluster Pathway

Stefano Da Vela, Giovanni Saudino, Francesca Lucarelli, Lucia Banci, Dmitri I. Svergun, Simone Ciofi-Baffoni

J Mol Biol. 2023 Aug 1; 435(15): 168154. doi: 10.1016/j.jmb.2023.168154

PMCID:: PMC10388178

Abstract Article

Select item 97353682.

Phenotypic continuum of NFU1‐related disorders

Rauan Kaiyrzhanov, Maha S. Zaki, Tracy Lau, Sambuddha Sen, Reza Azizimalamiri, Mina Zamani, Gözde Yeşil Sayin, Taru Hilander, Stephanie Efthymiou, Viorica Chelban, Ruth Brown, Kyle Thompson, Maria Irene Scarano, Jaya Ganesh, Kairgali Koneev, Ismail Musab Gülaçar, Richard Person, Dinara Sadykova, Yerdan Maidyrov, Tahereh Seifi, Aizhan Zadagali, Geneviève Bernard, Katrina Allis, Houda Zghal Elloumi, Amanda Lindy, Ehsan Taghiabadi, Sumit Verma, Rachel Logan, Brian Kirmse, Renkui Bai, Shaimaa M. Khalaf, Mohamed S. Abdel‐Hamid, Alireza Sedaghat, Gholamreza Shariati, Mahmoud Issa, Jawaher Zeighami, Hasnaa M. Elbendary, Garry Brown, Robert W. Taylor, Hamid Galehdari, Joseph J. Gleeson, Christopher J. Carroll, James A. Cowan, Andres Moreno‐De‐Luca, Henry Houlden, Reza Maroofian

Ann Clin Transl Neurol. 2022 Dec; 9(12): 2025–2035. Published online 2022 Oct 18. doi: 10.1002/acn3.51679

PMCID:: PMC9735368

Abstract Article PDF–1.4M

Select item 76892953.

Assembly of the [4Fe–4S] cluster of NFU1 requires the coordinated donation of two [2Fe–2S] clusters from the scaffold proteins, ISCU2 and ISCA1

Anshika Jain, Anamika Singh, Nunziata Maio, Tracey A Rouault

Hum Mol Genet. 2020 Oct 1; 29(19): 3165–3182. Published online 2020 Aug 8. doi: 10.1093/hmg/ddaa172

PMCID:: PMC7689295

Abstract Article PDF–2.1M

Select item 54668084.

Understanding the Molecular Basis for Multiple Mitochondrial Dysfunctions Syndrome 1 (MMDS1) - Impact of a Disease-Causing Gly208Cys Substitution on Structure and Activity of NFU1 in the Fe/S Cluster Biosynthetic Pathway

Christine Wachnowsky, Nathaniel A. Wesley, Insiya Fidai, J. A. Cowan

J Mol Biol. Author manuscript; available in PMC 2018 Mar 24.

Published in final edited form as: J Mol Biol. 2017 Mar 24; 429(6): 790–807. Published online 2017 Feb 1. doi: 10.1016/j.jmb.2017.01.021

PMCID:: PMC5466808

Abstract Article PDF–1.5M

Select item 51676775.

Iron–sulfur cluster exchange reactions mediated by the human Nfu protein

Christine Wachnowsky, Insiya Fidai, J. A. Cowan

J Biol Inorg Chem. Author manuscript; available in PMC 2017 Oct 1.

Published in final edited form as: J Biol Inorg Chem. 2016 Oct; 21(7): 825–836. Published online 2016 Aug 18. doi: 10.1007/s00775-016-1381-8

PMCID:: PMC5167677

Abstract Article PDF–1.3M

Select item 51665786.

Structural/Functional Properties of Human NFU1, an Intermediate [4Fe-4S] Carrier in Human Mitochondrial Iron-Sulfur Cluster Biogenesis

Kai Cai, Gaohua Liu, Ronnie O. Frederick, Rong Xiao, Gaetano T. Montelione, John L. Markley

Structure. Author manuscript; available in PMC 2016 Dec 19.

Published in final edited form as: Structure. 2016 Dec 6; 24(12): 2080–2091. Published online 2016 Nov 3. doi: 10.1016/j.str.2016.08.020

PMCID:: PMC5166578

Abstract Article PDF–1.6M

Select item 43338907.

New spastic paraplegia phenotype associated to mutation of NFU1

Davide Tonduti, Imen Dorboz, Apolline Imbard, Abdelhamid Slama, Audrey Boutron, Samia Pichard, Monique Elmaleh, Louis Vallée, Jean François Benoist, Heléne Ogier, Odile Boespflug-Tanguy

Orphanet J Rare Dis. 2015; 10: 13. Published online 2015 Feb 8. doi: 10.1186/s13023-015-0237-6

PMCID:: PMC4333890

Abstract Article PDF–545K

Select item 27665088.

Iron-Sulfur Cluster Biosynthesis: Characterization of a Molten-Globule Domain in Human NFU

Yushi Liu, J. A. Cowan

Biochemistry. Author manuscript; available in PMC 2010 Aug 11.

Published in final edited form as: Biochemistry. 2009 Aug 11; 48(31): 7512–7518. doi: 10.1021/bi9002524

PMCID:: PMC2766508

Abstract Article PDF–1.4M

Select item 106817259.

BOLA3 and NFU1 link mitoribosome iron–sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome

Hui Zhong, Alexandre Janer, Oleh Khalimonchuk, Hana Antonicka, Eric A Shoubridge, Antoni Barrientos

Nucleic Acids Res. 2023 Nov 27; 51(21): 11797–11812. Published online 2023 Oct 12. doi: 10.1093/nar/gkad842

PMCID:: PMC10681725

Abstract Article PDF–1.8M

Select item 569603010.

Understanding the Molecular Basis for Multiple Mitochondrial Dysfunctions Syndrome 1 (MMDS1): Impact of a Disease-Causing Gly189Arg Substitution on NFU1

Nathaniel A. Wesley, Christine Wachnowsky, Insiya Fidai, J. A. Cowan

FEBS J. Author manuscript; available in PMC 2018 Nov 1.

Published in final edited form as: FEBS J. 2017 Nov; 284(22): 3838–3848. Published online 2017 Oct 12. doi: 10.1111/febs.14271

PMCID:: PMC5696030

Abstract Article PDF–1.2M

Select item 321339811.

A Fatal Mitochondrial Disease Is Associated with Defective NFU1 Function in the Maturation of a Subset of Mitochondrial Fe-S Proteins

Aleix Navarro-Sastre, Frederic Tort, Oliver Stehling, Marta A. Uzarska, José Antonio Arranz, Mireia del Toro, M. Teresa Labayru, Joseba Landa, Aida Font, Judit Garcia-Villoria, Begoña Merinero, Magdalena Ugarte, Luis Gonzalez Gutierrez-Solana, Jaume Campistol, Angels Garcia-Cazorla, Julian Vaquerizo, Encarnació Riudor, Paz Briones, Orly Elpeleg, Antonia Ribes, Roland Lill

Am J Hum Genet. 2011 Nov 11; 89(5): 656–667. doi: 10.1016/j.ajhg.2011.10.005

PMCID:: PMC3213398

Abstract Article PDF–1.1M

Select item 318883512.

Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes

Jessie M. Cameron, Alexandre Janer, Valeriy Levandovskiy, Nevena MacKay, Tracey A. Rouault, Wing-Hang Tong, Isla Ogilvie, Eric A. Shoubridge, Brian H. Robinson

Am J Hum Genet. 2011 Oct 7; 89(4): 486–495. doi: 10.1016/j.ajhg.2011.08.011

PMCID:: PMC3188835

Abstract Article PDF–1.1M

Select item 577789613.

Regulation of Human Nfu Activity in Fe-S Cluster Delivery – Characterization of the Interaction between Nfu and the HSPA9/Hsc20 Chaperone Complex

Christine Wachnowsky, Yushi Liu, Taejin Yoon, J. A. Cowan

FEBS J. Author manuscript; available in PMC 2019 Jan 1.

Published in final edited form as: FEBS J. 2018 Jan; 285(2): 391–410. Published online 2017 Dec 29. doi: 10.1111/febs.14353

PMCID:: PMC5777896

Abstract Article PDF–2.1M

Select item 501455014.

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

Marta A Uzarska, Veronica Nasta, Benjamin D Weiler, Farah Spantgar, Simone Ciofi-Baffoni, Maria Rosaria Saviello, Leonardo Gonnelli, Ulrich Mühlenhoff, Lucia Banci, Roland Lill

eLife. 2016; 5: e16673. Published online 2016 Aug 17. doi: 10.7554/eLife.16673

PMCID:: PMC5014550

Abstract Article PDF–2.3M

Select item 992273415.

Patient-specific variants of NFU1/NFU-1 disrupt cholinergic signaling in a model of multiple mitochondrial dysfunctions syndrome 1

Peter A. Kropp, Philippa Rogers, Sydney E. Kelly, Rebecca McWhirter, Willow D. Goff, Ian M. Levitan, David M. Miller, III, Andy Golden

Dis Model Mech. 2023 Feb 1; 16(2): dmm049594. Published online 2023 Feb 1. doi: 10.1242/dmm.049594

PMCID:: PMC9922734

Abstract Article

Select item 18783916.

Subcellular compartmentalization of human Nfu, an iron–sulfur cluster scaffold protein, and its ability to assemble a [4Fe–4S] cluster

Wing-Hang Tong, Guy N. L. Jameson, Boi Hanh Huynh, Tracey A. Rouault

Proc Natl Acad Sci U S A. 2003 Aug 19; 100(17): 9762–9767. Published online 2003 Jul 28. doi: 10.1073/pnas.1732541100

PMCID:: PMC187839

Abstract Article PDF–448K

Select item 31087617.

Identification of Novel Human Genes Evolutionarily Conserved in Caenorhabditis elegans by Comparative Proteomics

Chun-Hung Lai, Chang-Yuan Chou, Lan-Yang Ch'ang, Chung-Shyan Liu, Wen-chang Lin

Genome Res. 2000 May; 10(5): 703–713. doi: 10.1101/gr.10.5.703

PMCID:: PMC310876

Abstract Article PDF–1.2M

Select item 662002818.

Mitochondrial ClpP-mediated proteolysis induces selective cancer cell lethality

Jo Ishizawa, Sarah F. Zarabi, R. Eric Davis, Ondrej Halgas, Takenobu Nii, Yulia Jitkova, Ran Zhao, Jonathan St-Germain, Lauren E. Heese, Grace Egan, Vivian R. Ruvolo, Samir H. Barghout, Yuki Nishida, Rose Hurren, Wencai Ma, Marcela Gronda, Todd Link, Keith Wong, Mark Mabanglo, Kensuke Kojima, Gautam Borthakur, Neil MacLean, John Man Chun Ma, Andrew B. Leber, Mark D. Minden, Walid Houry, Hagop Kantarjian, Martin Stogniew, Brian Raught, Emil F. Pai, Aaron D. Schimmer, Michael Andreeff

Cancer Cell. Author manuscript; available in PMC 2020 May 13.

Published in final edited form as: Cancer Cell. 2019 May 13; 35(5): 721–737.e9. Published online 2019 May 2. doi: 10.1016/j.ccell.2019.03.014

PMCID:: PMC6620028

Abstract Article PDF–2.9M

Select item 425650719.

The Functional Landscape of Hsp27 Reveals New Cellular Processes such as DNA Repair and Alternative Splicing and Proposes Novel Anticancer Targets

Maria Katsogiannou, Claudia Andrieu, Virginie Baylot, Anaïs Baudot, Nelson J. Dusetti, Odile Gayet, Pascal Finetti, Carmen Garrido, Daniel Birnbaum, François Bertucci, Christine Brun, Palma Rocchi

Mol Cell Proteomics. 2014 Dec; 13(12): 3585–3601. Published online 2014 Oct 1. doi: 10.1074/mcp.M114.041228

PMCID:: PMC4256507

Abstract Article PDF–3.9M

Select item 910630320.

Genome-wide CRISPR screens using isogenic cells reveal vulnerabilities conferred by loss of tumor suppressors

Xu Feng, Mengfan Tang, Merve Dede, Dan Su, Guangsheng Pei, Dadi Jiang, Chao Wang, Zhen Chen, Mi Li, Litong Nie, Yun Xiong, Siting Li, Jeong-Min Park, Huimin Zhang, Min Huang, Klaudia Szymonowicz, Zhongming Zhao, Traver Hart, Junjie Chen

Sci Adv. 2022 May; 8(19): eabm6638. Published online 2022 May 13. doi: 10.1126/sciadv.abm6638

PMCID:: PMC9106303

Abstract Article PDF–918K

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