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

5'-Deoxyadenosine

Formula: C10H13N5O3 (251.10183480000003)

CAS ID: 4754-39-6

Fe3+

Formula: Fe (55.934939)

CAS ID: 7439-89-6

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

hydrosulfide

Formula: HS (32.979896600000004)

CAS ID: 15035-72-0



Enzyme

EC Number name full name note
2.3.1.-
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
2.3.1.200 lipoyl amidotransferase [glycine cleavage system H]-N6-lipoyl-L-lysine:[lipoyl-carrier protein]-N6-L-lysine lipoyltransferase
2.8.1.8 lipoyl synthase [protein]-N6-(octanoyl)-L-lysine:an [Fe-S] cluster scaffold protein carrying a [4Fe-4S]2+ cluster sulfurtransferase


Proteins

Protein ID name full name