LIAS:2(4Fe-4S) transforms octanoyl-K107-GCSH to lipoyl-K107-GCSH

Stable Identifier
R-HSA-6793591
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Lipoate is an essential cofactor for five redox reactions; four in oxoacid dehydrogenases (active in energy metabolism and amino acid metabolism) and one in the glycine cleavage system (GCS). Lipoate synthesis in mitochondria requires three steps. In the second step, mitochondrial lipoyl synthase (LIAS) mediates the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to glycine cleavage system H protein (GCSH), transforming the octanoyl moiety to a lipoyl moiety. LIAS requires two 4Fe-4S clusters as cofactor which act as the sulfur donors in the reaction (Morikawa et al. 2001). Defects in LIAS causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation (Mayr et al. 2011).

Literature References
PubMed ID Title Journal Year
22152680 Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation

Mayr, JA, Zimmermann, FA, Fauth, C, Bergheim, C, Meierhofer, D, Radmayr, D, Zschocke, J, Koch, J, Sperl, W

Am. J. Hum. Genet. 2011
11389890 Do mammalian cells synthesize lipoic acid? Identification of a mouse cDNA encoding a lipoic acid synthase located in mitochondria

Morikawa, T, Yasuno, R, Wada, H

FEBS Lett. 2001
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
lipoate synthase activity of LIAS:2(4Fe-4S) [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
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Created