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Loss-of-function mutations in ISCA2 disrupt 4Fe-4S cluster machinery and cause a fatal leukodystrophy with hyperglycinemia and mtDNA depletion
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ISCA1 mutation in a patient with infantile-onset leukodystrophy causes defects in mitochondrial [4Fe-4S] proteins (vol 27, pg 2739, 2018)
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ISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo
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Biogenesis of cytosolic and nuclear iron-sulfur proteins and their role in genome stability
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Fe/S protein assembly gene IBA57 mutation causes hereditary spastic paraplegia
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ISCA2 mutation causes infantile neurodegenerative mitochondrial disorder
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Functional reconstitution of mitochondrial Fe/S cluster synthesis on Isu1 reveals the involvement of ferredoxin
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The mitochondrial carrier Rim2 co-imports pyrimidine nucleotides and iron
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BIOCHEMICAL JOURNAL (2013)
Arabidopsis thaliana Nfu2 Accommodates [2Fe-2S] or [4Fe-4S] Clusters and Is Competent for in Vitro Maturation of Chloroplast [2Fe-2S] and [4Fe-4S] Cluster-Containing Proteins
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Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy
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Monothiol Glutaredoxins Can Bind Linear [Fe3S4]+ and [Fe4S4]2+ Clusters in Addition to [Fe2S2]2+ Clusters: Spectroscopic Characterization and Functional Implications
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The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation
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Spectroscopic and Functional Characterization of Iron-Sulfur Cluster-Bound Forms of Azotobacter vinelandii NifIscA
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BIOCHEMISTRY (2012)
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The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation
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MOLECULAR BIOLOGY OF THE CELL (2012)
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AMERICAN JOURNAL OF HUMAN GENETICS (2011)
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Monothiol glutaredoxin Grx5 interacts with Fe-S scaffold proteins Isa1 and Isa2 and supports Fe-S assembly and DNA integrity in mitochondria of fission yeast
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Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts
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JOURNAL OF CLINICAL INVESTIGATION (2010)
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Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis
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JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Mitochondrial Iba57p is required for Fe/S cluster formation on aconitase and activation of radical SAM enzymes
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The human counterpart of zebrafish shiraz shows sideroblastic-like microcytic anemia and iron overload
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Deficiency of glutaredoxin 5 reveals Fe-S clusters are required for vertebrate haem synthesis
RA Wingert et al.
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EMBO JOURNAL (2003)
Grx5 is a mitochondrial glutaredoxin required for the activity of iron/sulfur enzymes
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MOLECULAR BIOLOGY OF THE CELL (2002)
A mitochondrial ferredoxin is essential for biogenesis of cellular iron-sulfur proteins
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