Names & Taxonomy
- Uniprot ID:
- O43766
- Entry Name:
- LIAS_HUMAN
- Status:
- reviewed
- Protein Names:
- Lipoyl synthase, mitochondrial (EC 2.8.1.8) (Lipoate synthase) (LS) (Lip-syn) (Lipoic acid synthase)
- Gene Names:
- LIAS LAS HUSSY-01
- Gene Names Orf:
- HUSSY-01
- Gene Names Primary:
- LIAS
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 372
- Sequence:
- MSLRCGDAARTLGPRVFGRYFCSPVRPLSSLPDKKKELLQNGPDLQDFVSGDLADRSTWDEYKGNLKRQKGERLRLPPWLKTEIPMGKNYNKLKNTLRNLNLHTVCEEARCPNIGECWGGGEYATATATIMLMGDTCTRGCRFCSVKTARNPPPLDASEPYNTAKAIAEWGLDYVVLTSVDRDDMPDGGAEHIAKTVSYLKERNPKILVECLTPDFRGDLKAIEKVALSGLDVYAHNVETVPELQSKVRDPRANFDQSLRVLKHAKKVQPDVISKTSIMLGLGENDEQVYATMKALREADVDCLTLGQYMQPTRRHLKVEEYITPEKFKYWEKVGNELGFHYTASGPLVRSSYKAGEFFLKNLVAKRKTKDL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- Catalyzes 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.
- Pathway:
- Protein modification; protein lipoylation via endogenous pathway; protein N(6)-(lipoyl)lysine from octanoyl-: step 2/2.
- Catalytic Activity:
- Protein N(6)-(octanoyl)lysine + 2 sulfur-(sulfur carrier) + 2 S-adenosyl-L-methionine + 2 reduced ferredoxin = protein N(6)-(lipoyl)lysine + 2 (sulfur carrier) + 2 L-methionine + 2 5'-deoxyadenosine + 2 oxidized ferredoxin.
- Cofactor:
- COFACTOR: Name= cluster; Xref=ChEBI:CHEBI:49883;
- Cross Reference Drug Bank:
- DB00166
- Gene Ontology Go:
- mitochondrial matrix
mitochondrion
4 iron, 4 sulfur cluster binding
lipoate synthase activity
metal ion binding
cellular nitrogen compound metabolic process
glyoxylate metabolic process
inflammatory response
lipoate biosynthetic process
neural tube closure
protein lipoylation
response to lipopolysaccharide
response to oxidative stress
small molecule metabolic process - Gene Ontology Biological Process:
- cellular nitrogen compound metabolic process
glyoxylate metabolic process
inflammatory response
lipoate biosynthetic process
neural tube closure
protein lipoylation
response to lipopolysaccharide
response to oxidative stress
small molecule metabolic process - Gene Ontology Molecular Function:
- 4 iron, 4 sulfur cluster binding
lipoate synthase activity
metal ion binding - Gene Ontology Cellular Component:
- mitochondrial matrix
mitochondrion - Keywords:
- 4Fe-4S
Alternative splicing
Complete proteome
Disease mutation
Iron
Iron-sulfur
Metal-binding
Mitochondrion
Reference proteome
S-adenosyl-L-methionine
Transferase
Transit peptide