Mitochondrial fatty acid synthesis pathway (Homo sapiens)

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4MECRMCATHKASMalonyl-ACPOctanoyl-ACPHTD2 ACACAMetaboliteHSD17B123-Ketoacyl-ACPMalonyl-CoAacetyl-CoAATPCO2ATPCO23-Hydroxyacyl-ACPtrans-2-Enoyl-ACPAcyl-ACPMedium/Long-chain fatty acidsAcetyl/Acyl-ACPNADPNADPHwaterHNADPNADPH


Schematic presentation of the sequence of events. The indicated abbreviations follow the nomenclature used for the human enzymes and proteins. ACC, acetyl-CoA carboxylase; MCT, malonyl-CoA transferase; ACP, acyl-carrier protein; KAS, ketoacyl synthase; KAR, ketoacyl reductase; HTD2, hydroxyacyl-thioester reductase type 2; ETR, enoyl-thioester reductase. [J. Kastaniotis et al 2010]

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Ontology Terms



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  1. Fernandez CA, Des Rosiers C; ''Modeling of liver citric acid cycle and gluconeogenesis based on 13C mass isotopomer distribution analysis of intermediates.''; J Biol Chem, 1995 PubMed Europe PMC
  2. Johnson JD, Muhonen WW, Lambeth DO; ''Characterization of the ATP- and GTP-specific succinyl-CoA synthetases in pigeon. The enzymes incorporate the same alpha-subunit.''; J Biol Chem, 1998 PubMed Europe PMC
  3. Sazanov LA, Jackson JB; ''Proton-translocating transhydrogenase and NAD- and NADP-linked isocitrate dehydrogenases operate in a substrate cycle which contributes to fine regulation of the tricarboxylic acid cycle activity in mitochondria.''; FEBS Lett, 1994 PubMed Europe PMC
  4. Hiltunen JK, Autio KJ, Schonauer MS, Kursu VA, Dieckmann CL, Kastaniotis AJ; ''Mitochondrial fatty acid synthesis and respiration.''; Biochim Biophys Acta, 2010 PubMed Europe PMC
  5. Des Rosiers C, Di Donato L, Comte B, Laplante A, Marcoux C, David F, Fernandez CA, Brunengraber H; ''Isotopomer analysis of citric acid cycle and gluconeogenesis in rat liver. Reversibility of isocitrate dehydrogenase and involvement of ATP-citrate lyase in gluconeogenesis.''; J Biol Chem, 1995 PubMed Europe PMC
  6. Liu TC, Kim H, Arizmendi C, Kitano A, Patel MS; ''Identification of two missense mutations in a dihydrolipoamide dehydrogenase-deficient patient.''; Proc Natl Acad Sci U S A, 1993 PubMed Europe PMC
  7. Greksák M, Lopes-Cardozo M, van den Bergh SG; ''Citrate synthesis in intact rat-liver mitochondria is irreversible.''; Eur J Biochem, 1982 PubMed Europe PMC
  8. Hoek JB, Rydström J; ''Physiological roles of nicotinamide nucleotide transhydrogenase.''; Biochem J, 1988 PubMed Europe PMC
  9. Des Rosiers C, Fernandez CA, David F, Brunengraber H; ''Reversibility of the mitochondrial isocitrate dehydrogenase reaction in the perfused rat liver. Evidence from isotopomer analysis of citric acid cycle intermediates.''; J Biol Chem, 1994 PubMed Europe PMC
  10. Sheu KF, Blass JP; ''The alpha-ketoglutarate dehydrogenase complex.''; Ann N Y Acad Sci, 1999 PubMed Europe PMC
  11. Krebs HA, Holzach O; ''The conversion of citrate into cis-aconitate and isocitrate in the presence of aconitase.''; Biochem J, 1952 PubMed Europe PMC
  12. Gabriel JL, Zervos PR, Plaut GW; ''Activity of purified NAD-specific isocitrate dehydrogenase at modulator and substrate concentrations approximating conditions in mitochondria.''; Metabolism, 1986 PubMed Europe PMC
  13. Comte B, Vincent G, Bouchard B, Benderdour M, Des Rosiers C; ''Reverse flux through cardiac NADP(+)-isocitrate dehydrogenase under normoxia and ischemia.''; Am J Physiol Heart Circ Physiol, 2002 PubMed Europe PMC
  14. Lawson AM, Chalmers RA, Watts RW; ''Urinary organic acids in man. I. Normal patterns.''; Clin Chem, 1976 PubMed Europe PMC
  15. Lambeth DO, Tews KN, Adkins S, Frohlich D, Milavetz BI; ''Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues.''; J Biol Chem, 2004 PubMed Europe PMC


106799view13:27, 17 September 2019MaintBotHMDB identifier normalization
97929view09:03, 25 June 2018MadeomugaModified description
97928view09:03, 25 June 2018MadeomugaAdd description
97924view08:47, 25 June 2018MadeomugaNew pathway

External references


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NameTypeDatabase referenceComment
3-Hydroxyacyl-ACP Metabolite
ACACAGeneProductENSG00000278540 (Ensembl)
ATPMetaboliteHMDB0000538 (HMDB) The ChEBI ID with the correct charge is: 30616
Acetyl/Acyl-ACP Metabolite
Acyl-ACP Metabolite
CO2MetaboliteHMDB0001967 (HMDB)
H Metabolite
HSD17B12GeneProductENSG00000149084 (Ensembl)
HTD2 GeneProductENSG00000255154 (Ensembl)
KASGeneProductENSG00000151093 (Ensembl)
MCATGeneProductENSG00000100294 (Ensembl)
MECRGeneProductENSG00000116353 (Ensembl)
Malonyl-ACP Metabolite
Malonyl-CoAMetaboliteHMDB0001175 (HMDB)
Medium/Long-chain fatty acids Metabolite
NADPHMetaboliteHMDB0000221 (HMDB) The ChEBI ID with the correct charge is: 57783
NADPMetaboliteHMDB0000217 (HMDB)
Octanoyl-ACP Metabolite
acetyl-CoAMetaboliteHMDB0001206 (HMDB) The ChEBI ID with the correct charge is: 57288
trans-2-Enoyl-ACP Metabolite
waterMetaboliteHMDB0002111 (HMDB)

Annotated Interactions

No annotated interactions

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