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Iscu iron-sulfur cluster assembly enzyme [ Mus musculus (house mouse) ]

Gene ID: 66383, updated on 9-Dec-2024

Summary

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Official Symbol
Iscuprovided by MGI
Official Full Name
iron-sulfur cluster assembly enzymeprovided by MGI
Primary source
MGI:MGI:1913633
See related
Ensembl:ENSMUSG00000025825 AllianceGenome:MGI:1913633
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
Nifu; ISCU2; Nifun; 2310020H20Rik
Summary
Enables protein homodimerization activity. Involved in [4Fe-4S] cluster assembly. Located in mitochondrion. Part of mitochondrial [2Fe-2S] assembly complex. Orthologous to human ISCU (iron-sulfur cluster assembly enzyme). [provided by Alliance of Genome Resources, Dec 2024]
Expression
Ubiquitous expression in adrenal adult (RPKM 128.4), kidney adult (RPKM 126.9) and 28 other tissues See more
Orthologs
human all
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Genomic context

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See Iscu in Genome Data Viewer
Location:
5 F; 5 55.55 cM
Exon count:
5
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 5 NC_000071.7 (113910479..113916349)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 5 NC_000071.6 (113772418..113778288)

Chromosome 5 - NC_000071.7Genomic Context describing neighboring genes Neighboring gene STARR-positive B cell enhancer ABC_E2171 Neighboring gene FIC domain containing Neighboring gene squamous cell carcinoma antigen recognized by T cells 3 Neighboring gene STARR-seq mESC enhancer starr_14052 Neighboring gene STARR-positive B cell enhancer ABC_E9260 Neighboring gene 40S ribosomal protein S28 pseudogene Neighboring gene transmembrane protein 119 Neighboring gene predicted gene, 42004 Neighboring gene STARR-positive B cell enhancer ABC_E3601

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. The role of zinc in the stability of the marginally stable IscU scaffold protein. Iannuzzi C, et al. Protein Sci, 2014 Sep. PMID 24917298, Free PMC Article
  2. Tissue-specific splicing of ISCU results in a skeletal muscle phenotype in myopathy with lactic acidosis, while complete loss of ISCU results in early embryonic death in mice. Nordin A, et al. Hum Genet, 2011 Apr. PMID 21165651
  3. Regulation of the cysteine desulfurase Nfs1 and the scaffold protein IscU in macrophages stimulated with interferon-gamma and lipopolysaccharide. Canal F, et al. Arch Biochem Biophys, 2007 Sep 1. PMID 17603005
  4. Regulation of iron homeostasis by the p53-ISCU pathway. Funauchi Y, et al. Sci Rep, 2015 Nov 12. PMID 26560363, Free PMC Article
  5. Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols. Parent A, et al. Nat Commun, 2015 Jan 19. PMID 25597503

See all (30) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. ISCU expression was decreased in the majority of human liver cancer tissues, and its reduced expression was significantly associated with p53 mutation.
    Title: Regulation of iron homeostasis by the p53-ISCU pathway.
  2. IscU is a marginally stable protein at low ionic strength to the point that undergoes cold denaturation at around -8 degrees C with a corresponding dramatic decrease of enthalpy, which is consistent with the fluxional nature of the protein.
    Title: The role of zinc in the stability of the marginally stable IscU scaffold protein.
  3. mTORC1 associates with ISCU and phosphorylates ISCU at serine 14. This phosphorylation stabilized ISCU protein.
    Title: Mammalian target of rapamycin complex 1 (mTORC1)-mediated phosphorylation stabilizes ISCU protein: implications for iron metabolism.
  4. Data show that complete loss of ISCU results in early embryonic death and confirm a fundamental role for ISCU in mammals.
    Title: Tissue-specific splicing of ISCU results in a skeletal muscle phenotype in myopathy with lactic acidosis, while complete loss of ISCU results in early embryonic death in mice.
  5. Results identify the iron-sulfur cluster assembly proteins (ISCU1/2) as direct targets for repression by the hypoxia-induced microRNA-210.
    Title: MicroRNA-210 controls mitochondrial metabolism during hypoxia by repressing the iron-sulfur cluster assembly proteins ISCU1/2.
  6. While IFN-gamma alone induced Nfs1 protein instability, LPS triggered a delayed decline of Nfs1, rather involving transcriptional events or mRNA instability.
    Title: Regulation of the cysteine desulfurase Nfs1 and the scaffold protein IscU in macrophages stimulated with interferon-gamma and lipopolysaccharide.
Submit: New GeneRIF Correction

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (1) 
  • Targeted (1) 

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables 2 iron, 2 sulfur cluster binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables ferrous iron binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables iron ion binding IEA
Inferred from Electronic Annotation
more info
  
enables iron-sulfur cluster binding IEA
Inferred from Electronic Annotation
more info
  
enables molecular adaptor activity ISO
Inferred from Sequence Orthology
more info
  
enables protein homodimerization activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables protein homodimerization activity ISO
Inferred from Sequence Orthology
more info
  
enables zinc ion binding ISO
Inferred from Sequence Orthology
more info
  
enables zinc ion binding ISS
Inferred from Sequence or Structural Similarity
more info
  
Process Evidence Code Pubs
involved_in [2Fe-2S] cluster assembly ISO
Inferred from Sequence Orthology
more info
  
involved_in [2Fe-2S] cluster assembly ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in [4Fe-4S] cluster assembly IDA
Inferred from Direct Assay
more info
 PubMed 
acts_upstream_of [4Fe-4S] cluster assembly ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_positive_effect [4Fe-4S] cluster assembly ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of [4Fe-4S] cluster assembly ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in intracellular iron ion homeostasis IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in intracellular iron ion homeostasis ISO
Inferred from Sequence Orthology
more info
  
involved_in intracellular iron ion homeostasis ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in iron-sulfur cluster assembly ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in iron-sulfur cluster assembly NAS
Non-traceable Author Statement
more info
 PubMed 
involved_in negative regulation of iron ion import across plasma membrane ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of aconitate hydratase activity ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of mitochondrial electron transport, NADH to ubiquinone ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
is_active_in cytoplasm IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in cytoplasm ISO
Inferred from Sequence Orthology
more info
  
located_in cytosol ISO
Inferred from Sequence Orthology
more info
  
part_of iron-sulfur cluster assembly complex NAS
Non-traceable Author Statement
more info
 PubMed 
part_of mitochondrial [2Fe-2S] assembly complex IDA
Inferred from Direct Assay
more info
 PubMed 
part_of mitochondrial [2Fe-2S] assembly complex ISO
Inferred from Sequence Orthology
more info
  
is_active_in mitochondrial matrix IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrion HDA PubMed 
located_in mitochondrion IDA
Inferred from Direct Assay
more info
 PubMed 
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion NAS
Non-traceable Author Statement
more info
 PubMed 

General protein information

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Preferred Names
iron-sulfur cluster assembly enzyme ISCU
Names
IscU iron-sulfur cluster scaffold homolog
iron-sulfur cluster assembly enzyme ISCU, mitochondrial
nifU-like N-terminal domain-containing protein

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001363317.1 → NP_001350246.1  iron-sulfur cluster assembly enzyme ISCU isoform 2

    Status: VALIDATED

    Source sequence(s)
    AC132939, AI851358, AK083247
    Conserved Domains (1) summary
    PRK11325
    Location:50 → 170
    PRK11325; scaffold protein; Provisional

  2. NM_025526.5 → NP_079802.1  iron-sulfur cluster assembly enzyme ISCU isoform 1 precursor

    See identical proteins and their annotated locations for NP_079802.1

    Status: VALIDATED

    Source sequence(s)
    AC132939
    Consensus CDS
    CCDS19553.1
    UniProtKB/Swiss-Prot
    Q4VBC7, Q8K344, Q9D7P6
    Related
    ENSMUSP00000026937.6, ENSMUST00000026937.12
    Conserved Domains (1) summary
    PRK11325
    Location:36 → 159
    PRK11325; scaffold protein; Provisional

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000071.7 Reference GRCm39 C57BL/6J

    Range
    113910479..113916349
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_030254754.1 → XP_030110614.1  iron-sulfur cluster assembly enzyme ISCU isoform X1

    Conserved Domains (1) summary
    PRK11325
    Location:117 → 221
    PRK11325; scaffold protein; Provisional

  2. XM_030254755.1 → XP_030110615.1  iron-sulfur cluster assembly enzyme ISCU isoform X2

    Conserved Domains (1) summary
    cl00528
    Location:50 → 151
    IscU_like; Iron-sulfur cluster scaffold-like proteins
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9D7P6.1 GenPept UniProtKB/Swiss-Prot:Q9D7P6

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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