LMPD Database

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LMP002266

UniProt Annotations

Entry Information
Gene Namelipoic acid synthetase
Protein EntryLIAS_HUMAN
UniProt IDQ6P5Q6
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=O43766-1; Sequence=Displayed; Name=2; IsoId=O43766-2; Sequence=VSP_047380, VSP_047381; Note=Gene prediction based on EST data.; Name=3; IsoId=O43766-3; Sequence=VSP_054764; Note=No experimental confirmation available. Gene prediction based on EST data.;
Catalytic ActivityProtein N(6)-(octanoyl)lysine + 2 sulfur- (sulfur carrier) + 2 S-adenosyl-L-methionine = protein N(6)- (lipoyl)lysine + 2 (sulfur carrier) + 2 L-methionine + 2 5'- deoxyadenosine.
CofactorName=[4Fe-4S] cluster; Xref=ChEBI
DiseasePyruvate dehydrogenase lipoic acid synthetase deficiency (PDHLD) [MIM
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.
PathwayProtein modification; protein lipoylation via endogenous pathway; protein N(6)-(lipoyl)lysine from octanoyl-[acyl-carrier- protein]: step 2/2.
SimilarityBelongs to the radical SAM superfamily. Lipoyl synthase family.
Subcellular LocationMitochondrion {ECO