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- W3048598112 abstract "Significance Synthesis of iron-sulfur clusters (ISC) and their insertion into apoproteins in eukaryotes requires the conserved, essential mitochondrial ISC and cytosolic Fe/S protein assembly machineries. Genetic mutations in most of the 18 different human ISC genes cause neurological, metabolic, and hematological diseases with often lethal outcome. Here, we have biochemically reconstituted the least-understood part of mitochondrial iron-sulfur protein assembly: The synthesis and insertion of [4Fe-4S] clusters. Apart from the in vivo-identified ISC factors (i.e., the [2Fe-2S] cluster donor GLRX5 and ISCA1–ISCA2–IBA57), this reaction specifically requires reduced ferredoxin FDX2, but not FDX1, for reductive fusion of [2Fe-2S] 2+ into [4Fe-4S] 2+ clusters. In contrast, [2Fe-2S] cluster transfer from GLRX5 to [2Fe-2S] target proteins occurs rapidly without any additional ISC factor." @default.
- W3048598112 created "2020-08-18" @default.
- W3048598112 creator A5005469733 @default.
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- W3048598112 creator A5073187685 @default.
- W3048598112 creator A5076790123 @default.
- W3048598112 creator A5090355454 @default.
- W3048598112 date "2020-08-12" @default.
- W3048598112 modified "2023-10-10" @default.
- W3048598112 title "Mitochondrial [4Fe-4S] protein assembly involves reductive [2Fe-2S] cluster fusion on ISCA1–ISCA2 by electron flow from ferredoxin FDX2" @default.
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- W3048598112 doi "https://doi.org/10.1073/pnas.2003982117" @default.
- W3048598112 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7456137" @default.
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- W3048598112 hasPublicationYear "2020" @default.
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