RGD Reference Report - Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery. - Rat Genome Database

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Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery.

Authors: Maio, N  Rouault, TA 
Citation: Maio N and Rouault TA, Biochim Biophys Acta. 2015 Jun;1853(6):1493-512. doi: 10.1016/j.bbamcr.2014.09.009. Epub 2014 Sep 19.
RGD ID: 11554190
Pubmed: PMID:25245479   (View Abstract at PubMed)
PMCID: PMC4366362   (View Article at PubMed Central)
DOI: DOI:10.1016/j.bbamcr.2014.09.009   (Journal Full-text)

Iron-sulfur (Fe-S) clusters are ancient, ubiquitous cofactors composed of iron and inorganic sulfur. The combination of the chemical reactivity of iron and sulfur, together with many variations of cluster composition, oxidation states and protein environments, enables Fe-S clusters to participate in numerous biological processes. Fe-S clusters are essential to redox catalysis in nitrogen fixation, mitochondrial respiration and photosynthesis, to regulatory sensing in key metabolic pathways (i.e. cellular iron homeostasis and oxidative stress response), and to the replication and maintenance of the nuclear genome. Fe-S cluster biogenesis is a multistep process that involves a complex sequence of catalyzed protein-protein interactions and coupled conformational changes between the components of several dedicated multimeric complexes. Intensive studies of the assembly process have clarified key points in the biogenesis of Fe-S proteins. However several critical questions still remain, such as: what is the role of frataxin? Why do some defects of Fe-S cluster biogenesis cause mitochondrial iron overload? How are specific Fe-S recipient proteins recognized in the process of Fe-S transfer? This review focuses on the basic steps of Fe-S cluster biogenesis, drawing attention to recent advances achieved on the identification of molecular features that guide selection of specific subsets of nascent Fe-S recipients by the cochaperone HSC20. Additionally, it outlines the distinctive phenotypes of human diseases due to mutations in the components of the basic pathway. This article is part of a Special Issue entitled: Fe/S proteins: Analysis, structure, function, biogenesis and diseases.



Molecular Pathway Annotations    Click to see Annotation Detail View

RGD Manual Annotations


  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
CIAO1Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
CIAO2AHumancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
CIAO2BHumancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
CIAO3Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
CIAPIN1Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
Ciao1Ratcytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO1 (Homo sapiens) RGD 
Ciao1Mousecytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO1 (Homo sapiens) RGD 
Ciao2aRatcytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO2A (Homo sapiens) RGD 
Ciao2aMousecytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO2A (Homo sapiens) RGD 
Ciao2bMousecytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO2B (Homo sapiens) RGD 
Ciao2bRatcytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO2B (Homo sapiens) RGD 
Ciao3Ratcytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO3 (Homo sapiens) RGD 
Ciao3Mousecytosolic iron-sulfur cluster protein assembly pathway   ISOCIAO3 (Homo sapiens) RGD 
Ciapin1Ratcytosolic iron-sulfur cluster protein assembly pathway   ISOCIAPIN1 (Homo sapiens) RGD 
Ciapin1Mousecytosolic iron-sulfur cluster protein assembly pathway   ISOCIAPIN1 (Homo sapiens) RGD 
MMS19Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
Mms19Ratcytosolic iron-sulfur cluster protein assembly pathway   ISOMMS19 (Homo sapiens) RGD 
Mms19Mousecytosolic iron-sulfur cluster protein assembly pathway   ISOMMS19 (Homo sapiens) RGD 
NDOR1Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
NUBP1Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
NUBP2Humancytosolic iron-sulfur cluster protein assembly pathway   TAS  RGD 
Ndor1Ratcytosolic iron-sulfur cluster protein assembly pathway   ISONDOR1 (Homo sapiens) RGD 
Ndor1Mousecytosolic iron-sulfur cluster protein assembly pathway   ISONDOR1 (Homo sapiens) RGD 
Nubp1Ratcytosolic iron-sulfur cluster protein assembly pathway   ISONUBP1 (Homo sapiens) RGD 
Nubp1Mousecytosolic iron-sulfur cluster protein assembly pathway   ISONUBP1 (Homo sapiens) RGD 
Nubp2Ratcytosolic iron-sulfur cluster protein assembly pathway   ISONUBP2 (Homo sapiens) RGD 
Nubp2Mousecytosolic iron-sulfur cluster protein assembly pathway   ISONUBP2 (Homo sapiens) RGD 
ABCB7Humanmitochondrial iron-sulfur cluster export pathway  TAS  RGD 
Abcb7Ratmitochondrial iron-sulfur cluster export pathway  ISOABCB7 (Homo sapiens) RGD 
Abcb7Mousemitochondrial iron-sulfur cluster export pathway  ISOABCB7 (Homo sapiens) RGD 
GFERHumanmitochondrial iron-sulfur cluster export pathway  TAS  RGD 
GferRatmitochondrial iron-sulfur cluster export pathway  ISOGFER (Homo sapiens) RGD 
GferMousemitochondrial iron-sulfur cluster export pathway  ISOGFER (Homo sapiens) RGD 
BOLA3Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
Bola3Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOBOLA3 (Homo sapiens) RGD 
Bola3Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOBOLA3 (Homo sapiens) RGD 
FDX1Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
FDX2Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
FDXRHumanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
Fdx1Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOFDX1 (Homo sapiens) RGD 
Fdx1Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOFDX1 (Homo sapiens) RGD 
Fdx2Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOFDX2 (Homo sapiens) RGD 
Fdx2Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOFDX2 (Homo sapiens) RGD 
FdxrRatmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOFDXR (Homo sapiens) RGD 
FdxrMousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOFDXR (Homo sapiens) RGD 
GLRX5Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
Glrx5Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOGLRX5 (Homo sapiens) RGD 
Glrx5Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOGLRX5 (Homo sapiens) RGD 
HSCBHumanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
HSPA9Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
HscbRatmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOHSCB (Homo sapiens) RGD 
HscbMousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOHSCB (Homo sapiens) RGD 
Hspa9Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOHSPA9 (Homo sapiens) RGD 
Hspa9Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOHSPA9 (Homo sapiens) RGD 
IBA57Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
ISCA1Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
ISCA2Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
ISCUHumanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
Iba57Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOIBA57 (Homo sapiens) RGD 
Iba57Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOIBA57 (Homo sapiens) RGD 
Isca1Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOISCA1 (Homo sapiens) RGD 
Isca1Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOISCA1 (Homo sapiens) RGD 
Isca2Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOISCA2 (Homo sapiens) RGD 
Isca2Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOISCA2 (Homo sapiens) RGD 
IscuRatmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOISCU (Homo sapiens) RGD 
IscuMousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOISCU (Homo sapiens) RGD 
LYRM4Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
Lyrm4Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISOLYRM4 (Homo sapiens) RGD 
Lyrm4Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISOLYRM4 (Homo sapiens) RGD 
NFS1Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
NFU1Humanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
NUBPLHumanmitochondrial iron-sulfur cluster protein biogenesis pathway   TAS  RGD 
Nfs1Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISONFS1 (Homo sapiens) RGD 
Nfs1Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISONFS1 (Homo sapiens) RGD 
Nfu1Ratmitochondrial iron-sulfur cluster protein biogenesis pathway   ISONFU1 (Homo sapiens) RGD 
Nfu1Mousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISONFU1 (Homo sapiens) RGD 
NubplRatmitochondrial iron-sulfur cluster protein biogenesis pathway   ISONUBPL (Homo sapiens) RGD 
NubplMousemitochondrial iron-sulfur cluster protein biogenesis pathway   ISONUBPL (Homo sapiens) RGD 
Objects Annotated

Genes (Rattus norvegicus)
Abcb7  (ATP binding cassette subfamily B member 7)
Bola3  (bolA family member 3)
Ciao1  (cytosolic iron-sulfur assembly component 1)
Ciao2a  (cytosolic iron-sulfur assembly component 2A)
Ciao2b  (cytosolic iron-sulfur assembly component 2B)
Ciao3  (cytosolic iron-sulfur assembly component 3)
Ciapin1  (cytokine induced apoptosis inhibitor 1)
Fdx1  (ferredoxin 1)
Fdx2  (ferredoxin 2)
Fdxr  (ferredoxin reductase)
Gfer  (growth factor, augmenter of liver regeneration)
Glrx5  (glutaredoxin 5)
Hscb  (HscB mitochondrial iron-sulfur cluster co-chaperone)
Hspa9  (heat shock protein family A (Hsp70) member 9)
Iba57  (iron-sulfur cluster assembly factor IBA57)
Isca1  (iron-sulfur cluster assembly 1)
Isca2  (iron-sulfur cluster assembly 2)
Iscu  (iron-sulfur cluster assembly enzyme)
Lyrm4  (LYR motif containing 4)
Mms19  (MMS19 homolog, cytosolic iron-sulfur assembly component)
Ndor1  (NADPH dependent diflavin oxidoreductase 1)
Nfs1  (NFS1 cysteine desulfurase)
Nfu1  (NFU1 iron-sulfur cluster scaffold)
Nubp1  (NUBP iron-sulfur cluster assembly factor 1)
Nubp2  (NUBP iron-sulfur cluster assembly factor 2)
Nubpl  (NUBP iron-sulfur cluster assembly factor like)

Genes (Mus musculus)
Abcb7  (ATP-binding cassette, sub-family B member 7)
Bola3  (bolA family member 3)
Ciao1  (cytosolic iron-sulfur protein assembly 1)
Ciao2a  (cytosolic iron-sulfur assembly component 2A)
Ciao2b  (cytosolic iron-sulfur assembly component 2B)
Ciao3  (cytosolic iron-sulfur assembly component 3)
Ciapin1  (cytokine induced apoptosis inhibitor 1)
Fdx1  (ferredoxin 1)
Fdx2  (ferredoxin 2)
Fdxr  (ferredoxin reductase)
Gfer  (growth factor, augmenter of liver regeneration)
Glrx5  (glutaredoxin 5)
Hscb  (HscB iron-sulfur cluster co-chaperone)
Hspa9  (heat shock protein 9)
Iba57  (IBA57 homolog, iron-sulfur cluster assembly)
Isca1  (iron-sulfur cluster assembly 1)
Isca2  (iron-sulfur cluster assembly 2)
Iscu  (iron-sulfur cluster assembly enzyme)
Lyrm4  (LYR motif containing 4)
Mms19  (MMS19 cytosolic iron-sulfur assembly component)
Ndor1  (NADPH dependent diflavin oxidoreductase 1)
Nfs1  (nitrogen fixation gene 1 (S. cerevisiae))
Nfu1  (NFU1 iron-sulfur cluster scaffold)
Nubp1  (nucleotide binding protein 1)
Nubp2  (nucleotide binding protein 2)
Nubpl  (nucleotide binding protein-like)

Genes (Homo sapiens)
ABCB7  (ATP binding cassette subfamily B member 7)
BOLA3  (bolA family member 3)
CIAO1  (cytosolic iron-sulfur assembly component 1)
CIAO2A  (cytosolic iron-sulfur assembly component 2A)
CIAO2B  (cytosolic iron-sulfur assembly component 2B)
CIAO3  (cytosolic iron-sulfur assembly component 3)
CIAPIN1  (cytokine induced apoptosis inhibitor 1)
FDX1  (ferredoxin 1)
FDX2  (ferredoxin 2)
FDXR  (ferredoxin reductase)
GFER  (growth factor, augmenter of liver regeneration)
GLRX5  (glutaredoxin 5)
HSCB  (HscB mitochondrial iron-sulfur cluster cochaperone)
HSPA9  (heat shock protein family A (Hsp70) member 9)
IBA57  (iron-sulfur cluster assembly factor IBA57)
ISCA1  (iron-sulfur cluster assembly 1)
ISCA2  (iron-sulfur cluster assembly 2)
ISCU  (iron-sulfur cluster assembly enzyme)
LYRM4  (LYR motif containing 4)
MMS19  (MMS19 homolog, cytosolic iron-sulfur assembly component)
NDOR1  (NADPH dependent diflavin oxidoreductase 1)
NFS1  (NFS1 cysteine desulfurase)
NFU1  (NFU1 iron-sulfur cluster scaffold)
NUBP1  (NUBP iron-sulfur cluster assembly factor 1, cytosolic)
NUBP2  (NUBP iron-sulfur cluster assembly factor 2, cytosolic)
NUBPL  (NUBP iron-sulfur cluster assembly factor, mitochondrial)


Additional Information