General Information of Drug Off-Target (DOT) (ID: OTX1VQ3P)

DOT Name Octanoyl-:protein N-octanoyltransferase LIPT2, mitochondrial (LIPT2)
Synonyms Lipoate-protein ligase B; Lipoyl/octanoyl transferase; Lipoyltransferase 2; EC 2.3.1.181; Octanoyl--protein N-octanoyltransferase
Gene Name LIPT2
Related Disease
Encephalopathy, neonatal severe, with lactic acidosis and brain abnormalities ( )
UniProt ID
LIPT2_HUMAN
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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EC Number
2.3.1.181
Sequence
MRQPAVRLVRLGRVPYAELLGLQDRWLRRLQAEPGIEAPSGTEAGALLLCEPAGPVYTAG
LRGGLTPEETARLRALGAEVRVTGRGGLATFHGPGQLLCHPVLDLRRLGLRLRMHVASLE
ACAVRLCELQGLQDARARPPPYTGVWLDDRKICAIGVRCGRHITSHGLALNCSTDLTWFE
HIVPCGLVGTGVTSLSKELQRHVTVEEVMPPFLVAFKEIYKCTLISEDSPN
Function
Catalyzes the transfer of endogenously produced octanoic acid from octanoyl-acyl-carrier-protein (octanoyl-ACP) onto the lipoyl domains of lipoate-dependent enzymes such as the protein H of the glycine cleavage system (GCSH). Lipoyl-ACP can also act as a substrate although octanoyl-ACP is likely to be the physiological substrate.
KEGG Pathway
Lipoic acid metabolism (hsa00785 )
Metabolic pathways (hsa01100 )
Biosynthesis of cofactors (hsa01240 )
Reactome Pathway
Glyoxylate metabolism and glycine degradation (R-HSA-389661 )
BioCyc Pathway
MetaCyc:G66-33937-MONOMER

Molecular Interaction Atlas (MIA) of This DOT

1 Disease(s) Related to This DOT
Disease Name Disease ID Evidence Level Mode of Inheritance REF
Encephalopathy, neonatal severe, with lactic acidosis and brain abnormalities DISKER8B Strong Autosomal recessive [1]
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Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DOT
2 Drug(s) Affected the Gene/Protein Processing of This DOT
Drug Name Drug ID Highest Status Interaction REF
Cisplatin DMRHGI9 Approved Cisplatin increases the expression of Octanoyl-:protein N-octanoyltransferase LIPT2, mitochondrial (LIPT2). [2]
Urethane DM7NSI0 Phase 4 Urethane decreases the expression of Octanoyl-:protein N-octanoyltransferase LIPT2, mitochondrial (LIPT2). [3]
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References

1 The Gene Curation Coalition: A global effort to harmonize gene-disease evidence resources. Genet Med. 2022 Aug;24(8):1732-1742. doi: 10.1016/j.gim.2022.04.017. Epub 2022 May 4.
2 Activation of AIFM2 enhances apoptosis of human lung cancer cells undergoing toxicological stress. Toxicol Lett. 2016 Sep 6;258:227-236.
3 Ethyl carbamate induces cell death through its effects on multiple metabolic pathways. Chem Biol Interact. 2017 Nov 1;277:21-32.