Cytosolic HSC20 integrates de novo iron-sulfur cluster biogenesis with the CIAO1-mediated transfer to recipients

Hum Mol Genet. 2018 Mar 1;27(5):837-852. doi: 10.1093/hmg/ddy004.

Abstract

Iron-sulfur (Fe-S) clusters are cofactors in hundreds of proteins involved in multiple cellular processes, including mitochondrial respiration, the maintenance of genome stability, ribosome biogenesis and translation. Fe-S cluster biogenesis is performed by multiple enzymes that are highly conserved throughout evolution, and mutations in numerous biogenesis factors are now recognized to cause a wide range of previously uncategorized rare human diseases. Recently, a complex formed of components of the cytoplasmic Fe-S cluster assembly (CIA) machinery, consisting of CIAO1, FAM96B and MMS19, was found to deliver Fe-S clusters to a subset of proteins involved in DNA metabolism, but it was unclear how this complex acquired its fully synthesized Fe-S clusters, because Fe-S clusters have been alleged to be assembled de novo solely in the mitochondrial matrix. Here, we investigated the potential role of the human cochaperone HSC20 in cytosolic Fe-S assembly and found that HSC20 assists Fe-S cluster delivery to cytosolic and nuclear Fe-S proteins. Cytosolic HSC20 (C-HSC20) mediated complex formation between components of the cytosolic Fe-S biogenesis pathway (ISC), including the primary scaffold, ISCU1, and the cysteine desulfurase, NFS1, and the CIA targeting complex, consisting of CIAO1, FAM96B and MMS19, to facilitate Fe-S cluster insertion into cytoplasmic and nuclear Fe-S recipients. Thus, C-HSC20 integrates initial Fe-S biosynthesis with the transfer activities of the CIA targeting system. Our studies demonstrate that a novel cytosolic pathway functions in parallel to the mitochondrial ISC to perform de novo Fe-S biogenesis, and to escort Fe-S clusters to cytoplasmic and nuclear proteins.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • Amino Acid Motifs
  • Carbon-Sulfur Lyases / genetics
  • Carbon-Sulfur Lyases / metabolism
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism
  • Cell Line
  • Cytoplasm / metabolism
  • Cytosol / metabolism*
  • DNA Polymerase III / genetics
  • DNA Polymerase III / metabolism
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Iron / metabolism*
  • Iron-Sulfur Proteins / genetics
  • Iron-Sulfur Proteins / metabolism
  • Metallochaperones / genetics
  • Metallochaperones / metabolism*
  • Molecular Chaperones / genetics
  • Molecular Chaperones / metabolism*
  • Multiprotein Complexes / metabolism
  • Mutagenesis
  • Sulfur / metabolism*
  • Zinc Fingers / genetics

Substances

  • CIAO1 protein, human
  • Carrier Proteins
  • HSCB protein, human
  • ISCU protein, human
  • Intracellular Signaling Peptides and Proteins
  • Iron-Sulfur Proteins
  • Metallochaperones
  • Molecular Chaperones
  • Multiprotein Complexes
  • NUBP1 protein, human
  • Sulfur
  • Iron
  • POLD1 protein, human
  • DNA Polymerase III
  • Carbon-Sulfur Lyases
  • NFS1 protein, human