Mitochondrial fatty acid synthesis pathway (Homo sapiens)
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Description
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
Bibliography
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- 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 Scholia
- Hiltunen JK, Schonauer MS, Autio KJ, Mittelmeier TM, Kastaniotis AJ, Dieckmann CL; ''Mitochondrial fatty acid synthesis type II: more than just fatty acids.''; J Biol Chem, 2009 PubMed Europe PMC Scholia
- Sheu KF, Blass JP; ''The alpha-ketoglutarate dehydrogenase complex.''; Ann N Y Acad Sci, 1999 PubMed Europe PMC Scholia
- 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 Scholia
- Hoek JB, Rydström J; ''Physiological roles of nicotinamide nucleotide transhydrogenase.''; Biochem J, 1988 PubMed Europe PMC Scholia
- Lawson AM, Chalmers RA, Watts RW; ''Urinary organic acids in man. I. Normal patterns.''; Clin Chem, 1976 PubMed Europe PMC Scholia
- 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 Scholia
- 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 Scholia
- 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 Scholia
- 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 Scholia
- 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 Scholia
- 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 Scholia
- 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 Scholia
- 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 Scholia
- Krebs HA, Holzach O; ''The conversion of citrate into cis-aconitate and isocitrate in the presence of aconitase.''; Biochem J, 1952 PubMed Europe PMC Scholia
- 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 Scholia
History
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External references
DataNodes
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Name | Type | Database reference | Comment |
---|---|---|---|
3-Hydroxyacyl-ACP | Metabolite | ||
3-Ketoacyl-ACP | Metabolite | ||
ACACA | GeneProduct | ENSG00000278540 (Ensembl) | |
ATP | Metabolite | HMDB0000538 (HMDB) | The ChEBI ID with the correct charge is: 30616 |
Acetyl/Acyl-ACP | Metabolite | ||
Acyl-ACP | Metabolite | ||
CO2 | Metabolite | HMDB0001967 (HMDB) | |
H | Metabolite | ||
HSD17B12 | GeneProduct | ENSG00000149084 (Ensembl) | |
HTD2 | GeneProduct | ENSG00000255154 (Ensembl) | |
KAS | GeneProduct | ENSG00000151093 (Ensembl) | |
MCAT | GeneProduct | ENSG00000100294 (Ensembl) | |
MECR | GeneProduct | ENSG00000116353 (Ensembl) | |
Malonyl-ACP | Metabolite | ||
Malonyl-CoA | Metabolite | HMDB0001175 (HMDB) | |
Medium/Long-chain fatty acids | Metabolite | ||
Metabolite | Metabolite | ||
NADPH | Metabolite | HMDB0000221 (HMDB) | The ChEBI ID with the correct charge is: 57783 |
NADP | Metabolite | HMDB0000217 (HMDB) | |
Octanoyl-ACP | Metabolite | ||
acetyl-CoA | Metabolite | HMDB0001206 (HMDB) | The ChEBI ID with the correct charge is: 57288 |
trans-2-Enoyl-ACP | Metabolite | ||
water | Metabolite | HMDB0002111 (HMDB) |
Annotated Interactions
No annotated interactions