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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
    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
    Physical map
    TRPC4AP 20q11.23 transient receptor potential cation channel, subfamily C, member 4 associated protein C20orf31 20q11.23 chromosome 20 open reading frame 31 PROCR 20q11.21 protein C receptor, endothelial (EPCR) MMP24 20q11.2 matrix metalloproteinase 24 (membrane-inserted) ITGB4BP 20q11.2 integrin beta 4 binding protein C20orf128 20q11.21 chromosome 20 open reading frame 128 C20orf44 20q11.23 chromosome 20 open reading frame 44 GDF5 20q11.2 growth differentiation factor 5 (cartilage-derived morphogenetic protein-1) CEP2 20pter-q12 centrosomal protein 2 LOC343705 20q11.23 similar to beta-galactoside alpha-2,3-sialyltransferase C20orf173 20q11.22 chromosome 20 open reading frame 173 SDBCAG84 20pter-q12 serologically defined breast cancer antigen 84 FER1L4 20q11.21 fer-1-like 4 (C. elegans) RPL37P1 11q13 ribosomal protein L37 pseudogene 1 LOC375095 20q11.23 similar to dJ309K20.1.1 (novel protein similar to dysferlin, isoform 1) SPAG4 20q11.2 sperm associated antigen 4 CPNE1 20q11.21 copine I RBM12 20q11.21 RNA binding motif protein 12 NFS1 20q11-q12 NFS1 nitrogen fixation 1 (S. cerevisiae) C20orf52 20q11.23 chromosome 20 open reading frame 52 RNPC2 20q11.21 RNA-binding region (RNP1, RRM) containing 2 C20orf53 20 chromosome 20 open reading frame 53 C20orf104 20q11.22-q11.23 chromosome 20 open reading frame 104 SCAND1 20q11.1-q11.23 SCAN domain containing 1 C20orf152 20q11.23 chromosome 20 open reading frame 152 HMG4L2 20q11.23 high-mobility group (nonhistone chromosomal) protein 4-like 2 EPB41L1 20q11.2-q12 erythrocyte membrane protein band 4.1-like 1
    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
    Type ubiquitous
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   moderately
    Digestiveesophagus   highly
     intestinesmall intestine  highly
     mouth   moderately
    Endocrineadrenal gland   predominantly
     parathyroid   highly
    Nervousbrain   moderately
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    at STAGE
    interspecies homolog to rattus Nfs1 (92.9 pc)
    homolog to murine Nfs1 (91.8 pc)
    intraspecies homolog to L-cysteine desulfurase
  • class V of pyridoxal-phosphate-dependent aminotransferases family
  • NIFS/ISCS subfamily
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
  • 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
    metabolism aminoacid
    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)
    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
    activated by ISCU and FXN that stimulate NFS1 and LYRM4 cysteine desulfurase activity
    corresponding disease(s) NFS1D
    Variant & Polymorphism
    Candidate gene
    Therapy target