Reactome | LIPT2 transfers octanoyl group to GCSH

LIPT2 transfers octanoyl group to GCSH

Stable Identifier

R-HSA-6793590

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix, mitochondrial inner membrane

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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