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The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism.
A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins.
MMS19 Assembles Iron-Sulfur Proteins Required for DNA Metabolism and Genomic Integrity
The function of MMS19 in the maturation of crucial components of DNA metabolism may explain the sensitivity of M MS19 mutants to DNA damage and the presence of extended telomeres.
Mitochondrial iron-sulfur protein biogenesis and human disease.
Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis
- A. Sheftel, O. Stehling, R. Lill
- Biology, ChemistryProceedings of the National Academy of Sciences
- 14 June 2010
It is concluded that mammals depend on two distinct mitochondrial ferredoxins for the specific production of either steroid hormones or heme A and Fe/S proteins.
The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation
The human mitochondrial proteins ISCA1, ISCA2, and IBA57 are essential for the generation of mitochondrial [4Fe-4S] proteins in a late step of Fe/S protein biogenesis. This process is important for…
Human Ind1, an Iron-Sulfur Cluster Assembly Factor for Respiratory Complex I
The human mitochondrial P-loop NTPase, designated huInd1, is identified as a new assembly factor for human respiratory complex I with a possible role in the delivery of one or more Fe/S clusters to complex I subunits.
Iron–sulfur proteins in health and disease
Human CIA2A-FAM96A and CIA2B-FAM96B integrate iron homeostasis and maturation of different subsets of cytosolic-nuclear iron-sulfur proteins.
Viperin is an iron‐sulfur protein that inhibits genome synthesis of tick‐borne encephalitis virus via radical SAM domain activity
The data suggest that viperin requires CIAO1 for [4Fe‐4S] cluster assembly, and acts through an enzymatic, Fe‐S cluster‐ and SAM‐dependent mechanism to inhibit viral RNA synthesis.