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Aliases for NFS1 Gene

Aliases for NFS1 Gene

  • NFS1 Cysteine Desulfurase 2 3
  • NIFS 3 4 6
  • Nitrogen Fixation 1 (S. Cerevisiae, Homolog) 2 3
  • EC 4 63
  • NFS1 Nitrogen Fixation 1 Homolog (S. Cerevisiae) 2
  • Nitrogen-Fixing Bacteria S-Like Protein 3
  • Cysteine Desulfurase, Mitochondrial 3
  • NFS1 Nitrogen Fixation 1 Homolog 3
  • HUSSY-08 3
  • IscS 3

External Ids for NFS1 Gene

Previous GeneCards Identifiers for NFS1 Gene

  • GC20U990032
  • GC20M033975
  • GC20M034925
  • GC20M034972
  • GC20M033720
  • GC20M034256
  • GC20M034220
  • GC20M031036
  • GC20M034255

Summaries for NFS1 Gene

Entrez Gene Summary for NFS1 Gene

  • Iron-sulfur clusters are required for the function of many cellular enzymes. The proteins encoded by this gene supply inorganic sulfur to these clusters by removing the sulfur from cysteine, creating alanine in the process. This gene uses alternate in-frame translation initiation sites to generate mitochondrial forms and cytoplasmic/nuclear forms. Selection of the alternative initiation sites is determined by the cytosolic pH. The encoded proteins belong to the class-V family of pyridoxal phosphate-dependent aminotransferases. Alternatively spliced transcript variants have been described. [provided by RefSeq, Nov 2010]

GeneCards Summary for NFS1 Gene

NFS1 (NFS1 Cysteine Desulfurase) is a Protein Coding gene. Diseases associated with NFS1 include severe neonatal lactic acidosis due to nfs1-isd11 complex deficiency and xanthinuria. Among its related pathways are Disease and Metabolism. GO annotations related to this gene include protein homodimerization activity and cysteine desulfurase activity. An important paralog of this gene is ENSG00000272897.

UniProtKB/Swiss-Prot for NFS1 Gene

  • Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor.

Gene Wiki entry for NFS1 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for NFS1 Gene

Genomics for NFS1 Gene

Regulatory Elements for NFS1 Gene

Epigenetics Products

  • DNA Methylation CpG Assay Predesigned for Pyrosequencing in human,mouse,rat

Genomic Location for NFS1 Gene

35,668,055 bp from pter
35,699,365 bp from pter
31,311 bases
Minus strand

Genomic View for NFS1 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for NFS1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for NFS1 Gene

Proteins for NFS1 Gene

  • Protein details for NFS1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Cysteine desulfurase, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B3KMA5
    • B4DXK9
    • E1P5R8
    • F5GYK5
    • Q6P0L8
    • Q9NTZ5
    • Q9Y481

    Protein attributes for NFS1 Gene

    457 amino acids
    Molecular mass:
    50196 Da
    Name=pyridoxal 5-phosphate; Xref=ChEBI:CHEBI:597326;
    Quaternary structure:
    • Binds ISCU/NIFUN. Forms a complex with LYRM4

    Alternative splice isoforms for NFS1 Gene


neXtProt entry for NFS1 Gene

Proteomics data for NFS1 Gene at MOPED

Post-translational modifications for NFS1 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for NFS1 Gene

Antibody Products

Domains for NFS1 Gene

Graphical View of Domain Structure for InterPro Entry



  • Q9Y697
  • Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. NifS/IscS subfamily.
genes like me logo Genes that share domains with NFS1: view

No data available for Gene Families for NFS1 Gene

Function for NFS1 Gene

Molecular function for NFS1 Gene

UniProtKB/Swiss-Prot BiophysicochemicalProperties:
Kinetic parameters: KM=434.75 uM for L-cysteine {ECO:0000269 PubMed:18650437}; Note=Kinetic parameter was determined for the protein lacking the 55 N-terminal amino acids and in a complex with LYRM4.;
UniProtKB/Swiss-Prot CatalyticActivity:
L-cysteine + acceptor = L-alanine + S-sulfanyl-acceptor
UniProtKB/Swiss-Prot Function:
Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor.

Enzyme Numbers (IUBMB) for NFS1 Gene

Gene Ontology (GO) - Molecular Function for NFS1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0003824 catalytic activity --
GO:0005515 protein binding IPI 16527810
GO:0016829 lyase activity --
GO:0030170 pyridoxal phosphate binding IEA --
GO:0031071 cysteine desulfurase activity TAS --
genes like me logo Genes that share ontologies with NFS1: view

Animal Model Products

miRNA for NFS1 Gene

miRTarBase miRNAs that target NFS1

No data available for Phenotypes , Animal Models , Transcription Factor Targeting and HOMER Transcription for NFS1 Gene

Localization for NFS1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for NFS1 Gene

Isoform Mitochondrial: Mitochondrion.
Isoform Cytoplasmic: Cytoplasm. Nucleus.

Subcellular locations from

Jensen Localization Image for NFS1 Gene COMPARTMENTS Subcellular localization image for NFS1 gene
Compartment Confidence
mitochondrion 5
nucleus 5
cytosol 4

Gene Ontology (GO) - Cellular Components for NFS1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005634 nucleus IDA 16847322
GO:0005654 nucleoplasm IDA --
GO:0005730 nucleolus IDA --
GO:0005737 cytoplasm IDA --
GO:0005739 mitochondrion TAS 9885568
genes like me logo Genes that share ontologies with NFS1: view

Pathways for NFS1 Gene

SuperPathways for NFS1 Gene

Superpath Contained pathways
1 Defective BTD causes biotidinase deficiency
2 Disease
3 Metabolism
4 Sulfur relay system
5 Mitochondrial Iron-Sulfur Cluster Biogenesis
genes like me logo Genes that share pathways with NFS1: view

Gene Ontology (GO) - Biological Process for NFS1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000096 sulfur amino acid metabolic process TAS 9885568
GO:0006461 protein complex assembly TAS 9885568
GO:0006520 cellular amino acid metabolic process --
GO:0006534 cysteine metabolic process IEA --
GO:0006766 vitamin metabolic process TAS --
genes like me logo Genes that share ontologies with NFS1: view

Compounds for NFS1 Gene

(4) HMDB Compounds for NFS1 Gene

Compound Synonyms Cas Number PubMed IDs
  • Armco iron
  • (2S)-2-Aminopropanoate
  • (+)-2-Amino-3-mercaptopropionic acid
Pyridoxal 5'-phosphate
  • Apolon B6

(3) Drugbank Compounds for NFS1 Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
  • (2S)-2-Aminopropanoic acid
56-41-7 target
  • (R)-2-Amino-3-mercaptopropanoic acid
52-90-4 target
Pyridoxal Phosphate
  • Codecarboxylase
54-47-7 target cofactor

(12) Novoseek inferred chemical compound relationships for NFS1 Gene

Compound -log(P) Hits PubMed IDs
iron-sulfur 85.8 5
moco 78.5 2
cysteine 74.2 30
sulfur 73.4 12
molybdopterin 70.4 2
genes like me logo Genes that share compounds with NFS1: view

Transcripts for NFS1 Gene

Unigene Clusters for NFS1 Gene

NFS1 nitrogen fixation 1 homolog (S. cerevisiae):
Representative Sequences:

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for NFS1

Primer Products

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for NFS1 Gene

No ASD Table

Relevant External Links for NFS1 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for NFS1 Gene

mRNA expression in normal human tissues for NFS1 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Protein differential expression in normal tissues for NFS1 Gene

This gene is overexpressed in Adrenal (9.6) and Skin (6.3).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for NFS1 Gene

SOURCE GeneReport for Unigene cluster for NFS1 Gene Hs.194692

mRNA Expression by UniProt/SwissProt for NFS1 Gene

Tissue specificity: Predominantly expressed in heart and skeletal muscle. Also found in brain, liver and pancreas
genes like me logo Genes that share expressions with NFS1: view

Expression partners for NFS1 Gene

In Situ Assay Products

No data available for mRNA differential expression in normal tissues for NFS1 Gene

Orthologs for NFS1 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for NFS1 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia NFS1 35
  • 92.85 (n)
  • 93 (a)
NFS1 36
  • 93 (a)
(Canis familiaris)
Mammalia NFS1 35
  • 92.49 (n)
  • 94.09 (a)
NFS1 36
  • 94 (a)
(Mus musculus)
Mammalia Nfs1 35
  • 88.03 (n)
  • 94.84 (a)
Nfs1 16
Nfs1 36
  • 91 (a)
(Pan troglodytes)
Mammalia NFS1 35
  • 99.64 (n)
  • 99.34 (a)
NFS1 36
  • 99 (a)
(Rattus norvegicus)
Mammalia Nfs1 35
  • 89.12 (n)
  • 95.31 (a)
(Monodelphis domestica)
Mammalia -- 36
  • 87 (a)
(Ornithorhynchus anatinus)
Mammalia -- 36
  • 66 (a)
(Gallus gallus)
Aves LOC100858210 35
  • 73.94 (n)
  • 85.21 (a)
NFS1 36
  • 82 (a)
NFS1 36
  • 82 (a)
(Anolis carolinensis)
Reptilia -- 36
  • 82 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia nfs1 35
  • 73.63 (n)
  • 85.32 (a)
Str.16278 35
African clawed frog
(Xenopus laevis)
Amphibia Xl.6508 35
(Danio rerio)
Actinopterygii nfs1 35
  • 73.71 (n)
  • 82.34 (a)
nfs1 36
  • 75 (a)
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.6217 35
fruit fly
(Drosophila melanogaster)
Insecta CG12264 37
  • 67 (a)
CG12264 35
  • 66.17 (n)
  • 78.3 (a)
CG12264 36
  • 69 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP009094 35
  • 67.75 (n)
  • 80.05 (a)
(Caenorhabditis elegans)
Secernentea B0205.6 37
  • 65 (a)
B0205.6 35
  • 63.84 (n)
  • 70.07 (a)
B0205.6 36
  • 68 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AAL013W 35
  • 61.25 (n)
  • 66.25 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0D04928g 35
  • 59.25 (n)
  • 64.75 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes NFS1 35
  • 60.42 (n)
  • 67.5 (a)
NFS1 36
  • 56 (a)
NFS1 38
thale cress
(Arabidopsis thaliana)
eudicotyledons NFS1 35
  • 62.18 (n)
  • 67.08 (a)
(Glycine max)
eudicotyledons Gma.2991 35
(Hordeum vulgare)
Liliopsida Hv.4410 35
(Oryza sativa)
Liliopsida Os.9367 35
Os09g0338400 35
  • 65.34 (n)
  • 72.07 (a)
(Triticum aestivum)
Liliopsida Ta.10246 35
(Zea mays)
Liliopsida Zm.4660 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.10386 35
bread mold
(Neurospora crassa)
Ascomycetes NCU04636 35
  • 64.83 (n)
  • 70.75 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes nfs1 35
  • 61.15 (n)
  • 67.88 (a)
Species with no ortholog for NFS1:
  • Actinobacteria (Mycobacterium tuberculosis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)

Evolution for NFS1 Gene

Gene Tree for NFS1 (if available)
Gene Tree for NFS1 (if available)

Paralogs for NFS1 Gene

Paralogs for NFS1 Gene

genes like me logo Genes that share paralogs with NFS1: view

Variants for NFS1 Gene

Sequence variations from dbSNP and Humsavar for NFS1 Gene

SNP ID Clin Chr 20 pos Sequence Context AA Info Type MAF
rs2180280 -- 35,669,429(+) GGGAG(C/T)GCTCC utr-variant-3-prime, nc-transcript-variant
rs2378391 -- 35,680,534(+) AAAGA(A/T)TGGCC intron-variant
rs2425069 -- 35,673,458(+) tgCTC(C/T)AGAAA intron-variant
rs2425070 -- 35,681,469(+) AAGGC(A/G)GACGG intron-variant
rs3056722 -- 35,680,952(+) TATCT(-/CCA)CCAAT intron-variant

Structural Variations from Database of Genomic Variants (DGV) for NFS1 Gene

Variant ID Type Subtype PubMed ID
nsv526188 CNV Gain 19592680
esv2671511 CNV Deletion 23128226

Relevant External Links for NFS1 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for NFS1 Gene

Disorders for NFS1 Gene

MalaCards: The human disease database

MalaCards: The human disease database.

Search for NFS1 Gene in MalaCards »

(2) Diseases for NFS1 Gene including...

(1) University of Copenhagen DISEASES for NFS1 Gene

Relevant External Links for NFS1

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with NFS1: view

No data available for OMIM , UniProtKB/Swiss-Prot , Novoseek inferred disease relationships and Genatlas for NFS1 Gene

Publications for NFS1 Gene

  1. Targeting of a human iron-sulfur cluster assembly enzyme, nifs, to different subcellular compartments is regulated through alternative AUG utilization. (PMID: 9885568) Land T. … Rouault T.A. (Mol. Cell 1998) 2 3 4 23
  2. Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells. (PMID: 11060020) Tong W.-H. … Rouault T. (EMBO J. 2000) 3 4 23
  3. Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis. (PMID: 19454487) Shi Y. … Rouault T.A. (Hum. Mol. Genet. 2009) 4 23
  4. Characterization of 16 novel human genes showing high similarity to yeast sequences. (PMID: 11124703) Stanchi F. … Valle G. (Yeast 2001) 3 4
  5. Roles of the mammalian cytosolic cysteine desulfurase, ISCS, and scaffold protein, ISCU, in iron-sulfur cluster assembly. (PMID: 16527810) Li K. … Rouault T.A. (J. Biol. Chem. 2006) 3 23

Products for NFS1 Gene

Sources for NFS1 Gene

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