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   Articles with "iscu" as a keyword



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

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recommendations! Published in 2018 at "Biochimie"

DOI: 10.1016/j.biochi.2018.07.012

Abstract: Human de novo iron-sulfur (Fe-S) assembly complex consists of cysteine desulfurase NFS1, accessory protein ISD11, acyl carrier protein ACP, scaffold protein ISCU, and allosteric activator frataxin (FXN). FXN binds the NFS1-ISD11-ACP-ISCU complex (SDAU), to activate… read more here.

Keywords: zinc; wild type; iscu; desulfurase activity ... See more keywords
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ISCU interacts with NFU1, and ISCU[4Fe-4S] transfers its Fe-S cluster to NFU1 leading to the production of holo-NFU1

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recommendations! Published in 2020 at "Journal of structural biology"

DOI: 10.1016/j.jsb.2020.107491

Abstract: NFU1 is a late-acting factor in the biogenesis of human mitochondrial iron-sulfur proteins. Mutations in NFU1 are associated with genetic diseases such as multiple mitochondrial dysfunctions syndrome 1 (MMDS1) that involve defects in mitochondrial [4Fe-4S]… read more here.

Keywords: transfers cluster; 4fe transfers; iscu; iscu interacts ... See more keywords

Mechanistic Insights into IscU Conformation Regulation for Fe-S Cluster Biogenesis Revealed by Variable Temperature Electrospray Ionization Native Ion Mobility Mass Spectrometry.

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recommendations! Published in 2022 at "Biochemistry"

DOI: 10.1021/acs.biochem.2c00429

Abstract: Iron-sulfur (Fe-S) cluster (ISC) cofactors are required for the function of many critical cellular processes. In the ISC Fe-S cluster biosynthetic pathway, IscU assembles Fe-S cluster intermediates from iron, electrons, and inorganic sulfur, which is… read more here.

Keywords: variable temperature; iscu; cluster; electrospray ionization ... See more keywords

Autosomal dominant myopathy caused by a novel ISCU variant

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recommendations! Published in 2025 at "Frontiers in Genetics"

DOI: 10.3389/fgene.2025.1605440

Abstract: Hereditary myopathy with lactic acidosis due to Iron-Sulfur Cluster Assembly Enzyme (ISCU) deficiency is a rare disorder of energy metabolism characterized clinically by myopathy with exercise intolerance, and biochemically by deficiencies of skeletal muscle mitochondrial… read more here.

Keywords: variant; myopathy caused; iscu; dominant myopathy ... See more keywords