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NameCysteine desulfurase, mitochondrial
Synonyms
  • 2.8.1.7
  • NIFS
Gene NameNFS1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0000301|Cysteine desulfurase, mitochondrial
MLLRAAWRRAAVAVTAAPGPKPAAPTRGLRLRVGDRAPQSAVPADTAAAPEVGPVLRPLY
MDVQATTPLDPRVLDAMLPYLINYYGNPHSRTHAYGWESEAAMERARQQVASLIGADPRE
IIFTSGATESNNIAIKGVARFYRSRKKHLITTQTEHKCVLDSCRSLEAEGFQVTYLPVQK
SGIIDLKELEAAIQPDTSLVSVMTVNNEIGVKQPIAEIGRICSSRKVYFHTDAAQAVGKI
PLDVNDMKIDLMSISGHKIYGPKGVGAIYIRRRPRVRVEALQSGGGQERGMRSGTVPTPL
VVGLGAACEVAQQEMEYDHKRISKLSERLIQNIMKSLPDVVMNGDPKHHYPGCINLSFAY
VEGESLLMALKDVALSSGSACTSASLEPSYVLRAIGTDEDLAHSSIRFGIGRFTTEEEVD
YTVEKCIQHVKRLREMSPLWEMVQDGIDLKSIKWTQH
Number of residues457
Molecular Weight50195.21
Theoretical pI8.45
GO Classification
Functions
  • iron-sulfur cluster binding
  • pyridoxal phosphate binding
  • metal ion binding
  • cysteine desulfurase activity
  • protein homodimerization activity
Processes
  • [2Fe-2S] cluster assembly
  • iron incorporation into metallo-sulfur cluster
  • Mo-molybdopterin cofactor biosynthetic process
  • molybdopterin cofactor biosynthetic process
  • sulfur amino acid metabolic process
  • protein complex assembly
  • small molecule metabolic process
  • vitamin metabolic process
  • water-soluble vitamin metabolic process
Components
  • cytoplasm
  • mitochondrion
  • nucleus
  • mitochondrial matrix
  • cytosol
  • nucleolus
  • nucleoplasm
  • intracellular membrane-bounded organelle
General FunctionPyridoxal phosphate binding
Specific FunctionCatalyzes 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.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID24042327
UniProtKB IDQ9Y697
UniProtKB Entry NameNFS1_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0009970|Cysteine desulfurase, mitochondrial (NFS1)
ATGCTGCTCCGAGCCGCTTGGAGGCGGGCGGCAGTGGCGGTGACAGCGGCTCCAGGGCCG
AAGCCCGCGGCGCCCACTCGGGGGCTGCGCCTGCGCGTTGGAGACCGTGCTCCTCAGTCT
GCGGTTCCCGCAGATACAGCCGCTGCCCCGGAGGTGGGGCCAGTGCTGCGACCTCTCTAT
ATGGATGTGCAAGCTACAACTCCTCTGGACCCCCGGGTGCTTGATGCCATGCTCCCTTAC
CTAATCAACTACTATGGGAACCCACACTCCCGGACACATGCTTATGGCTGGGAGAGTGAG
GCAGCCATGGAACGTGCTCGTCAGCAAGTAGCATCTCTGATTGGAGCTGATCCTCGTGAG
ATCATTTTTACTAGTGGTGCTACTGAATCCAACAACATAGCAATTAAGGAACTAGAGGCT
GCTATCCAGCCAGATACTAGCCTGGTGTCAGTCATGACTGTGAACAATGAGATTGGAGTG
AAGCAGCCTATTGCAGAAATAGGGCGGATTTGCAGTTCCAGAAAGGTATATTTCCATACT
GATGCAGCCCAGGCTGTTGGAAAAATCCCACTTGATGTCAATGACATGAAAATTGATCTC
ATGAGCATTAGTGGTCACAAAATCTACGGTCCCAAAGGGGTTGGTGCCATCTACATCCGT
CGCCGGCCCCGTGTGCGTGTGGAGGCCCTGCAGAGTGGAGGGGGGCAGGAGCGGGGTATG
CGGTCTGGGACAGTGCCCACACCCTTAGTGGTGGGGCTGGGGGCTGCGTGTGAGGTGGCA
CAGCAAGAGATGGAGTATGACCACAAGCGAATCTCAAAGTTGTCAGAGCGGCTGATACAG
AATATAATGAAGAGCCTTCCAGATGTGGTGATGAATGGGGACCCTAAGCACCATTATCCC
GGCTGTATCAACCTCTCCTTTGCATATGTGGAAGGGGAAAGTCTGCTGATGGCACTGAAG
GACGTTGCCTTATCCTCAGGGAGTGCCTGCACCTCTGCATCCCTGGAGCCCTCTTATGTG
CTTAGAGCAATTGGCACTGATGAGGATTTAGCGCACTCTTCTATCAGGTTTGGAATTGGC
CGCTTCACTACAGAGGAGGAAGTGGACTACACAGTGGAGAAATGCATTCAGCATGTGAAG
CGTCTTCGAGAAATGAGCCCTCTCTGGGAGATGGTTCAGGATGGCATTGACCTCAAGAGC
ATCAAGTGGACCCAACACTAG
GenBank Gene IDAF097025
GeneCard IDNot Available
GenAtlas IDNFS1
HGNC IDHGNC:15910
Chromosome Location20
Locus20q11.22
References
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