Protein lipoylation

Arribas-Carreira L, Dallabona C, Swanson MA, Farris J, Østergaard E, Tsiakas K, Hempel M, Aquaviva-Bourdain C, Koutsoukos S, Stence NV, Magistrati M, Spector EB, Kronquist K, Christensen M, Karstensen HG, Feichtinger RG, Achleitner MT, Lawrence Merritt Ii J, Pérez B, Ugarte M, Grünewald S, Riela AR, Julve N, Arnoux JB, Haldar K, Donnini C, Santer R, Lund AM, Mayr JA, Rodriguez-Pombo P, Van Hove JLK. Pathogenic variants in GCSH encoding the moonlighting H-protein cause combined nonketotic hyperglycinemia and lipoate deficiency. Hum Mol Genet. 2023 Mar 06;32(6):917–33. PMID: 36190515; PMCID: PMC9990993.; Cronan JE. Progress in the Enzymology of the Mitochondrial Diseases of Lipoic Acid Requiring Enzymes. Front. Genet. 2020 May 21;11(). doi: 10.3389/fgene.2020.00510.; Cao X, Hong Y, Zhu L, Hu Y, Cronan JE. Development and retention of a primordial moonlighting pathway of protein modification in the absence of selection presents a puzzle. Proc Natl Acad Sci U S A. 2018 Jan 23;115(4):647–55. PMID: 29339506; PMCID: PMC5789953.; Schonauer MS, Kastaniotis AJ, Kursu VAS, Hiltunen JK, Dieckmann CL. Lipoic Acid Synthesis and Attachment in Yeast Mitochondria. Journal of Biological Chemistry. 2009 Aug;284(35):23234–42. doi: 10.1074/jbc.m109.015594.

Metabolites

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5



Enzyme

EC Number name full name note
2.3.1.12 dihydrolipoyllysine-residue acetyltransferase acetyl-CoA:enzyme N6-(dihydrolipoyl)lysine S-acetyltransferase
2.3.1.181 lipoyl(octanoyl) transferase octanoyl-[acyl-carrier protein]:protein N-octanoyltransferase
2.3.1.61 dihydrolipoyllysine-residue succinyltransferase succinyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-succinyltransferase
6.3.1.20 lipoate-protein ligase [lipoyl-carrier protein]-L-lysine:lipoate ligase (AMP-forming)


Proteins

Protein ID name full name
O13629 aim22 Putative lipoate-protein ligase A
O36017 LIPB_SCHPO Probable octanoyltransferase
O59816 lat1 Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
O94681 kgd2 Probable dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial