(S)-carnitine

Three steps

• CHEBI:17769Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine:(acceptor) oxidoreductase

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 1 morecytosol

• trans-urocanateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by SLC6A14, HALextracellular space, cytosol

• 5-hydroxy-L-tryptophanReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by SLC6A14, TPH1, TPH2extracellular space, cytosol

• a 1,2-diacyl-sn-glycerolReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacyl-sn-glycerol + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 6 morecytosol, Golgi apparatus

• a triacylglycerolReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 8 morecytosol, Golgi apparatus

• 4-methyl-2-oxopentanoateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by SLC6A14, BCAT2extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 3-oxopropanoic acidReach from here →
  1. Carnitine Transport by Atb0
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC6A14, SLC6A6, ABATextracellular space, cytosol, mitochondrial matrix

• 3-phenylpyruvateReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by SLC6A14, GOT2, TATextracellular space, cytosol, mitochondrial matrixOne of 5 routes

• fumaric acidReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of fumarate and sulfate (cytosol to mitochondria)

  Catalysed by SLC6A14, SLC26A6, SLC25A10extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• spermine monoaldehydeReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. primary-amine oxidase (spermine)

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 4 morecytosolOne of 3 routes

• CHEBI:18132Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Acetyl-CoA:choline O-acetyltransferase
  3. ATP:choline phosphotransferase

  Catalysed by CPT1A, CPT1C, CPT1B, CHAT and 2 morecytosol, nucleus

• dodecanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 11 morecytosolOne of 14 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrix

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 4 morecytosolOne of 3 routes

• 2-phenylethylamineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by SLC6A14, DDCextracellular space, cytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Glutamine by Atb0 Transporter
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by SLC6A14, PFASextracellular space, cytosol

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 8 morecytosol

• beta-D-xylosyl-(1<->1')-N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. Utilized Transport
  2. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate
  3. N-(9Z-octadecenoyl)-sphing-4-enine + UDP-alpha-D-xylose = beta-D-xylosyl-(1<->1')-N-(9Z-octadecenoyl)-sphing-4-enine + UDP + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreendoplasmic reticulum, cytosol

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 7 morecytosol

• ophthalmateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrix

• S-methylmercury-L-cysteineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 6 routes

• 2-aminoisobutanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC7A10cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 11-cis-retinyl esterReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, DGAT1 and 4 morecytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. ATP citrate lyase (citrate)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by CPT1A, CPT1C, CPT1B, ACLY and 1 morecytosol

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrixOne of 2 routes

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, CROT and 2 morecytosol, mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 9 morecytosol

• 1-(9Z-octadecenoyl)-3-octadecanoyl-sn-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 3-octadecanoyl-sn-glycerol + (9Z)-octadecenoyl-CoA = 1-(9Z-octadecenoyl)-3-octadecanoyl-sn-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 5 moreendoplasmic reticulum, cytosol

• 1-octanoyl-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-octanoylglycerol + (9Z)-octadecenoyl-CoA = 1-octanoyl-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 5 moreendoplasmic reticulum, cytosol

• 1-dodecanoyl-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-dodecanoylglycerol + (9Z)-octadecenoyl-CoA = 1-dodecanoyl-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 6 moreendoplasmic reticulum, cytosol

• 1-octadecanoyl-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-octadecanoylglycerol + (9Z)-octadecenoyl-CoA = 1-octadecanoyl-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 6 moreendoplasmic reticulum, cytosol

• 1-(9Z,12Z,15Z-octadecatrienoyl)-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-(9Z,12Z,15Z-octadecatrienoyl)-glycerol + (9Z)-octadecenoyl-CoA = 1-(9Z,12Z,15Z-octadecatrienoyl)-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 6 moreendoplasmic reticulum, cytosol

• 1-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-glycerol + (9Z)-octadecenoyl-CoA = 1-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 5 moreendoplasmic reticulum, cytosol

• 1-tetradecanoyl-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-tetradecanoylglycerol + (9Z)-octadecenoyl-CoA = 1-tetradecanoyl-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 5 moreendoplasmic reticulum, cytosol

• 1-O-(9Z-octadecyl)-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-O-(9Z-octadecenyl)-glycerol + (9Z)-octadecenoyl-CoA = 1-O-(9Z-octadecyl)-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 10 moreendoplasmic reticulum, cytosol

• 1-decanoyl-3-(9Z-octadecenoyl)-glycerolReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-decanoylglycerol + (9Z)-octadecenoyl-CoA = 1-decanoyl-3-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC27A2, ACSL3, ACSL1, ACSBG2 and 5 moreendoplasmic reticulum, cytosol

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrixOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by CPT1A, CPT1C, CPT1B, NAGS and 1 morecytosol, mitochondrial matrix

• 20-O-(beta-D-glucuronosyl)-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Utilized Transport
  2. arachidonic acid:oxygen 1-oxidoreductase
  3. 20-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = 20-O-(beta-D-glucuronosyl)-(5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP + H(+)

  Catalysed by CYP1A1, CYP1A2, CYP2E1, CYP3A4 and 26 moreendoplasmic reticulum, cytosol

• 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(3'-(9Z-octadecenoyl)-1'-sn-glycerol)Reach from here →
  1. Utilized Transport
  2. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol) + H2O = sn-glycero-3-phospho-(1'-sn-glycerol) + (9Z)-octadecenoate + H(+)
  3. 2 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol) = 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(3'-(9Z-octadecenoyl)-1'-sn-glycerol) + sn-glycero-3-phospho-(1'-sn-glycerol)

  Catalysed by ABHD12, CLN5endoplasmic reticulum, cytosol

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, ELOVL7 and 6 morecytosol

• a 2-hexadecanoyl-6-alpha-D-glucosaminy-1-(1,2-diacyl-sn-glycero-3-phospho)-1D-myo-inositolReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. a 6-(alpha-D-glucosaminyl)-1-(1,2-diacyl-sn-glycero-3-phospho)-1D-myo-inositol + hexadecanoyl-CoA = a 2-hexadecanoyl-6-alpha-D-glucosaminy-1-(1,2-diacyl-sn-glycero-3-phospho)-1D-myo-inositol + CoA

  Catalysed by CPT2, CPT1A, CPT1B, PIGWperoxisome

• (2E,5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + H2O2

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moreendoplasmic reticulum, cytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 10 morecytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by CPT1A, CPT1C, CPT1B, HADHA and 4 morecytosol, mitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by CPT1A, CPT1C, CPT1B, HADHA and 2 morecytosol, mitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by CPT1A, CPT1C, CPT1B, HADHA and 4 morecytosol, mitochondrial matrix

• (2E,4Z,7Z)-decatrienoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial
  3. Fatty Acid Beta Oxidation (C10:2->C10:3), Mitochondrial

  Catalysed by CPT1A, CPT1C, CPT1B, ECHS1 and 3 morecytosol, mitochondrial matrixOne of 3 routes

• docosanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 9 morecytosolOne of 16 routes

• octanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by CPT1A, CPT1C, CPT1B, CROT and 1 morecytosol, mitochondrial matrixOne of 2 routes