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FLASH GENE
Symbol NFS1 contributors: mct/npt - updated : 09-05-2014
HGNC name NFS1 nitrogen fixation 1 homolog (S. cerevisiae)
HGNC id 15910
Corresponding disease
NFS1D NFS1 deficiency
Location 20q11.22      Physical location : 34.256.610 - 34.287.274
Synonym name
  • cysteine desulfurase
  • nitrogen-fixing bacteria S-like protein
    • Synonym symbol(s) NIFS, HUSSY-8, IscS
    EC.number 2.8.1.7
    DNA
    TYPE functioning gene
    STRUCTURE 30.66 kb     13 Exon(s)    1 Copie(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    13 - 2385 47 457 located in mitochondria 2008 1865043
  • generated by initiation of the first AUG of the NFS1 transcript and contains a mitochondrial targeting signal at the N terminus that undergoes cleavage to yield a mature mitochondrial protein of 47 kDa in size
    • - - - 44 - in the cytosol and in the nucleus 2008 1865043
  • generated by initiation of translation at the second in-frame AUG lacks the first 60 residues of the mitochondrial precursor form
  • has an additional role in the cytosol and sulfurates MOCS3 for the biosynthesis of the molybdenum cofactor
  • required for cell viability and for post-translational modification of tRNAs in the nucleus
    • 12 - 2232 - 406 - 2008 1865043
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   moderately
    Digestiveesophagus   highly
     intestinesmall intestine  highly
     liver    
     mouth   moderately
    Endocrineadrenal gland   predominantly
     pancreas    
     parathyroid   highly
    Nervousbrain   moderately
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumcardiac  
    Muscularstriatumskeletal  
    Nervouscentral   
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
    HOMOLOGY
    interspecies homolog to rattus Nfs1 (92.9 pc)
    homolog to murine Nfs1 (91.8 pc)
    intraspecies homolog to L-cysteine desulfurase
    Homologene
    FAMILY
  • class V of pyridoxal-phosphate-dependent aminotransferases family
  • NIFS/ISCS subfamily
    • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus
    text
  • colocalization of NFS1 and MOCS3 in the cytosol
    • basic FUNCTION
  • catalyzing the removal of elemental sulfur from cysteine to produce alanine
  • supplying the inorganic sulfur for iron- sulfur (Fe/S) clusters
  • acts as direct sulfur donor for the biosynthesis of Moco in the cytosol
  • cysteine desulfurase responsible for providing sulfur for cluster formation and is required for the increased Isu stability occurring after disruption of cluster formation on or transfer from ISCU
  • plays a crucial role in the mitochondrial iron-sulfur cluster biosynthesis and in the thiomodification of mitochondrial and cytosolic tRNAs
  • in mitochondria, involved in Fe/S cluster biosynthesis
  • cysteine desulfurase, which serves as the sulfur donor for cluster assembly
  • cysteine desulfurase that is implicated in respiratory chain function and iron maintenance by initiating Fe-S cluster biosynthesis
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid
    signaling
    sulfur amino acid metabolism
    a component
  • form a tight complex with LYRM4, thus stabilizing NFS1 and preventing its aggregation
  • complex SDU and SDUF comprised of NFS1, LYRM4, and ISCU and NFS1, LYRM4, ISCU, and FXN, respectively (SDUF is capable of synthesizing Fe/S cluster)
    • INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • pyridoxal phosphate
    • protein
  • binding to NIFUN
  • can act as a sulfur donor for MOCS3, involved in Moco biosynthesis
  • interacting with LYRM4 (acts as an adaptor of NFS1 by forming a tight complex that helps NFS1 to fulfill the cysteine desulfurase function in the Fe/S cluster biosynthesis either in mitochondria or in the nuclear/cytosolic compartment of cells) (Shi 2009)
  • is able to transfer sulfur to the C-terminal domain of MOCS3, a cytosolic protein involved in molybdenum cofactor biosynthesis and tRNA thiolation
  • binds preferentially to the D-state of ISCU as does the NFS1-LYRM4 complex
  • NFS1 alone could catalyze Fe-S cluster assembly on ISCU and that the addition of LYRM4 increased the Fe-S cluster assembly rate
  • LYRM4 has a profound effect on the oligomeric state of NFS1
  • ISCU suppressor mimics the frataxin effects on NFS1, explaining the bypassing activity
  • FXN accelerates persulfide formation on NFS1 and favors a helix-to-coil interconversion on ISCU that facilitates the transfer of sulfur from NFS1 to ISCU as an initial step in Fe-S cluster biosynthesis
    • cell & other
    REGULATION
    activated by ISCU and FXN that stimulate NFS1 and LYRM4 cysteine desulfurase activity
    ASSOCIATED DISORDERS
    corresponding disease(s) NFS1D
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS