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FLASH GENE
Symbol NFU1 contributors: mct - updated : 27-06-2023
HGNC name NFU1 iron-sulfur cluster scaffold homolog (S. cerevisiae)
HGNC id 16287
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestivemouth   highly
Nervousnerve   highly
Respiratorylung    
Urinarykidney    
Visualeye   highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / Hematopoieticbone marrow   
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period fetal
Text pancreas
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • an N-terminal domain with a fully folded regular tertiary structure
  • a NifU-like domain at the C-terminus, having a pair of redox active cysteines and thioredoxin-like activity by both binding to and mediating persulfide bond cleavage of sulfur-loaded IscS, the sulfide donor for [2Fe-2S] cluster assembly on ISU-type scaffold proteins
  • HOMOLOGY
    Homologene
    FAMILY NIFU family
    CATEGORY storage
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm
    basic FUNCTION
  • involved in respiratory chain biogenis
  • may be functioning in nitrogen fixation
  • implicated in cellular iron-sulfur cluster biosynthesis
  • functionally competent reducing agent for cysteinyl persulfide bond cleavage, releasing inorganic sulfide for incorporation into the ISU-bound [2Fe-2S] cluster, a reactivity that might be facilitated by the flexibility of the C-terminal domain
  • play essential roles in the production of [Fe-S] centers for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the respiratory chain complexes
  • NFU1- (and BOLA3-) dependent assembled clusters are directed to complex I and lipoate synthesis
  • could function as an alternate scaffold to ISCU for assembly of [Fe-S] proteins, thus providing parallel pathways
  • functions as a late-acting maturation factor for a subset of mitochondrial Fe-S proteins
  • role of mitochondrial NFU1 late in the Fe-S protein biogenesis pathway is similar to the assumed function of the mitochondrial P loop NTPase IND1, which also transiently binds an Fe-S cluster and is specifically required for assembly of respiratory complex I
  • is a mitochondrial iron-sulfur scaffold protein, involved in iron-sulfur assembly and transfer to complex II and LAS (lipoic acid synthase)
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • laforin (laforin-binding partner)
  • NFU1, BOLA3, ISCA2 and IBA57 operate in the maturation of [4Fe-4S] clusters, and IBA57 seems to require BOLA3, NFU1 and ISCA2 for its stability
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MMDFS
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS