LIPT2 transfers octanoyl group to GCSH

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial lipoyltransferase 2 (LIPT2) transfers an octanoyl group bound to the acyl-carrier protein NDUFAB1 (ACP) to mitochondrial glycine cleavage system H protein (GCSH) at lysine 107 (Schonauer et al. 2009; Cao et al., 2018). The octanoyl moiety is provided by mitochondrial fatty acid synthesis pathways (Feng et al., 2009; Witkowski et al., 2011). Mutations in LIPT2 can cause severe neonatal encephalopathy by lipoate deficiency of mitochondrial dehydrogenase complexes (NELABA. MIM:617668; Habarou et al., 2017).
Literature References
PubMed ID Title Journal Year
29987032 Protein moonlighting elucidates the essential human pathway catalyzing lipoic acid assembly on its cognate enzymes

Hu, Z, Zhu, L, Cao, X, Song, X, Cronan, JE

Proc Natl Acad Sci U S A 2018
19221180 Down-regulation of mitochondrial acyl carrier protein in mammalian cells compromises protein lipoylation and respiratory complex I and results in cell death

Witkowski, A, Feng, D, Smith, S

J Biol Chem 2009
21846720 Mammalian ACSF3 protein is a malonyl-CoA synthetase that supplies the chain extender units for mitochondrial fatty acid synthesis

Witkowski, A, Thweatt, J, Smith, S

J Biol Chem 2011
28757203 Biallelic Mutations in LIPT2 Cause a Mitochondrial Lipoylation Defect Associated with Severe Neonatal Encephalopathy

Meitinger, T, Chadefaux-Vekemans, B, Nitschké, P, Korenke, GC, Marquardt, I, Pontoizeau, C, Bole-Feysot, C, Feichtinger, RG, Strom, TM, Boutron, A, Mayr, JA, Laroche, C, Hamel, Y, Kölker, S, Chrétien, D, Eisermann, M, Madrange, M, Habarou, F, Rötig, A, Goudin, N, Rodenburg, RJ, Nemazanyy, I, Grisel, C, Delahodde, A, Lebigot, E, Ottolenghi, C, Busiah, K, de Lonlay, P, Boddaert, N, Haack, TB, Barouki, R, Prokisch, H

Am J Hum Genet 2017
19570983 Lipoic acid synthesis and attachment in yeast mitochondria

Dieckmann, CL, Kursu, VA, Schonauer, MS, Hiltunen, JK, Kastaniotis, AJ

J. Biol. Chem. 2009
Catalyst Activity

lipoyl(octanoyl) transferase activity of LIPT2 [mitochondrial matrix]

Orthologous Events
Cross References
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