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NameLipoyl synthase, mitochondrial
Synonyms
  • 2.8.1.8
  • LAS
  • Lip-syn
  • Lipoate synthase
  • Lipoic acid synthase
  • LS
Gene NameLIAS
OrganismHuman
Amino acid sequence
>lcl|BSEQ0037190|Lipoyl synthase, mitochondrial
MSLRCGDAARTLGPRVFGRYFCSPVRPLSSLPDKKKELLQNGPDLQDFVSGDLADRSTWD
EYKGNLKRQKGERLRLPPWLKTEIPMGKNYNKLKNTLRNLNLHTVCEEARCPNIGECWGG
GEYATATATIMLMGDTCTRGCRFCSVKTARNPPPLDASEPYNTAKAIAEWGLDYVVLTSV
DRDDMPDGGAEHIAKTVSYLKERNPKILVECLTPDFRGDLKAIEKVALSGLDVYAHNVET
VPELQSKVRDPRANFDQSLRVLKHAKKVQPDVISKTSIMLGLGENDEQVYATMKALREAD
VDCLTLGQYMQPTRRHLKVEEYITPEKFKYWEKVGNELGFHYTASGPLVRSSYKAGEFFL
KNLVAKRKTKDL
Number of residues372
Molecular Weight41910.695
Theoretical pI8.77
GO Classification
Functions
  • metal ion binding
  • lipoate synthase activity
  • 4 iron, 4 sulfur cluster binding
Processes
  • protein lipoylation
  • response to lipopolysaccharide
  • neural tube closure
  • lipoate biosynthetic process
  • response to oxidative stress
  • inflammatory response
Components
  • mitochondrion
General FunctionMetal ion binding
Specific FunctionCatalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID23958222
UniProtKB IDO43766
UniProtKB Entry NameLIAS_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0020609|Lipoyl synthase, mitochondrial (LIAS)
ATGTCTCTACGCTGCGGGGATGCAGCCCGCACCCTGGGGCCCCGGGTATTTGGGAGATAT
TTTTGCAGCCCAGTCAGACCGTTAAGCTCCTTGCCAGATAAAAAAAAGGAACTCCTACAG
AATGGACCAGACCTTCAAGATTTTGTATCTGGTGATCTTGCAGACAGGAGCACCTGGGAT
GAATATAAAGGAAACCTAAAACGCCAGAAAGGAGAAAGGTTAAGACTACCTCCATGGCTA
AAGACAGAGATTCCCATGGGGAAAAATTACAATAAACTGAAAAATACTTTGCGGAATTTA
AATCTCCATACAGTATGTGAGGAAGCTCGATGTCCCAATATTGGAGAGTGTTGGGGAGGT
GGAGAATATGCCACCGCCACAGCCACGATCATGTTGATGGGTGACACATGTACAAGAGGT
TGCAGATTTTGTTCTGTTAAGACTGCAAGAAATCCTCCTCCACTGGATGCCAGTGAGCCC
TACAATACTGCAAAGGCAATTGCAGAATGGGGTCTGGATTATGTTGTCCTGACATCTGTG
GATCGAGATGATATGCCTGATGGGGGAGCTGAACACATTGCAAAGACCGTATCATATTTA
AAGGAAAGTAAGGTTCGTGATCCTCGGGCCAATTTTGATCAGTCCCTACGTGTACTGAAA
CATGCCAAGAAGGTTCAGCCTGATGTTATTTCTAAAACATCTATAATGTTGGGTTTAGGC
GAGAATGATGAGCAAGTATATGCAACAATGAAAGCACTTCGTGAGGCAGATGTAGACTGC
TTGACTTTAGGACAATATATGCAGCCAACAAGGCGTCACCTTAAGGTTGAAGAATATATT
ACTCCTGAAAAATTCAAATACTGGGAAAAAGTAGGAAATGAACTTGGATTTCATTATACT
GCAAGTGGCCCTTTGGTGCGTTCTTCATATAAAGCAGGTGAATTTTTCCTGAAAAATCTA
GTGGCTAAAAGAAAAACAAAAGACCTCTAA
GenBank Gene IDBC023635
GeneCard IDNot Available
GenAtlas IDLIAS
HGNC IDHGNC:16429
Chromosome Location4
Locus4p14
References
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