Protein: Acyl-coenzyme A synthetase ACSM1, mitochondrial (EC 6.2.1.2) (Acyl-CoA synthetase medium-chain family member 1) (Benzoate--CoA ligase) (EC 6.2.1.25) (Butyrate--CoA ligase 1) (Butyryl-coenzyme A synthetase 1) (Lipoate-activating enzyme) (Middle-chain acyl-CoA synthetase 1) (Xenobiotic/medium-chain fatty acid-CoA ligase HXM-B) (Q08AH1)
Organism: Homo sapiens (Human)
Function: Catalyzes the activation of fatty acids by CoA to produce an acyl-CoA, the first step in fatty acid metabolism. Capable of activating medium-chain fatty acids (e.g. butyric (C4) to decanoic (C10) acids), and certain carboxylate-containing xenobiotics, e.g. benzoate. Also catalyzes the activation of lipoate to lipoyl-nucleoside monophosphate (By similarity). Activates lipoate with GTP at a 1000-fold higher rate than with ATP and activates both (R)- and (S)-lipoate to the respective lipoyl-GMP, with a preference for (R)-lipoate (By similarity).
Subcellular location: Mitochondrion matrix {ECO:0000250|UniProtKB:Q91VA0}. Mitochondrion {ECO:0000250|UniProtKB:Q91VA0}.
Sequence: MQWLMRFRTLWGIHKSFHNIHPAPSQLRCRSLSEFGAPRWNDYEVPEEFNFASYVLDYWAQKEKEGKRGPNPAFWWVNGQGDEVKWSFREMGDLTRRVANVFTQTCGLQQGDHLALMLPRVPEWWLVAVGCMRTGIIFIPATILLKAKDILYRLQLSKAKGIVTIDALASEVDSIASQCPSLKTKLLVSDHSREGWLDFRSLVKSASPEHTCVKSKTLDPMVIFFTSGTTGFPKMAKHSHGLALQPSFPGSRKLRSLKTSDVSWCLSDSGWIVATIWTLVEPWTAGCTVFIHHLPQFDTKVIIQTLLKYPINHFWGVSSIYRMILQQDFTSIRFPALEHCYTGGEVVLPKDQEEWKRRTGLLLYENYGQSETGLICATYWGMKIKPGFMGKATPPYDVQVIDDKGSILPPNTEGNIGIRIKPVRPVSLFMCYEGDPEKTAKVECGDFYNTGDRGKMDEEGYICFLGRSDDIINASGYRIGPAEVESALVEHPAVAESAVVGSPDPIRGEVVKAFIVLTPQFLSHDKDQLTKELQQHVKSVTAPYKYPRKVEFVSELPKTITGKIERKELRKKETGQM
InterPro domains: - IPR042099: ANL, N-terminal domain (homologous_superfamily) [39-469]
- IPR051087: Mitochondrial Acyl-CoA Synthetase Medium-Chain (family) [1-576]
- IPR000873: AMP-dependent synthetase/ligase domain (domain) [72-419]
- IPR045851: AMP-binding enzyme, C-terminal domain superfamily (homologous_superfamily) [470-575]
- IPR025110: AMP-binding enzyme, C-terminal domain (domain) [483-563]
- IPR020845: AMP-binding, conserved site (conserved_site) [223-234]
Interaction partners: - Lipoyl amidotransferase LIPT1, mitochondrial
- Short-chain specific acyl-CoA dehydrogenase, mitochondrial
- Solute carrier family 22 member 16
- Guanylate-binding protein 5
- Hydroxysteroid dehydrogenase-like protein 2
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016874 (ligase activity), GO:0005515 (protein binding), GO:0016877 (ligase activity, forming carbon-sulfur bonds), GO:0016878 (acid-thiol ligase activity), GO:0016405 (CoA-ligase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0044281 (small molecule metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0071704 (organic substance metabolic process), GO:0044238 (primary metabolic process), GO:1901360 (organic cyclic compound metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0006629 (lipid metabolic process), GO:0006139 (nucleobase-containing compound metabolic process), GO:0046483 (heterocycle metabolic process), GO:0055086 (nucleobase-containing small molecule metabolic process), GO:0019637 (organophosphate metabolic process), GO:0006725 (cellular aromatic compound metabolic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0043603 (amide metabolic process), GO:0006790 (sulfur compound metabolic process), GO:1901135 (carbohydrate derivative metabolic process), GO:0035383 (thioester metabolic process), GO:0006793 (phosphorus metabolic process), GO:0006753 (nucleoside phosphate metabolic process), GO:0006637 (acyl-CoA metabolic process), GO:0019693 (ribose phosphate metabolic process), GO:0006796 (phosphate-containing compound metabolic process), GO:1901568 (fatty acid derivative metabolic process), GO:0072521 (purine-containing compound metabolic process), GO:0009259 (ribonucleotide metabolic process), GO:0009117 (nucleotide metabolic process), GO:0035337 (fatty-acyl-CoA metabolic process), GO:0006163 (purine nucleotide metabolic process), GO:0034032 (purine nucleoside bisphosphate metabolic process), GO:0033865 (nucleoside bisphosphate metabolic process), GO:0009150 (purine ribonucleotide metabolic process), GO:0033875 (ribonucleoside bisphosphate metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)