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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
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