(S)-carnitine

Three steps

• sphing-4-enine 1-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 5 morecytosol, Golgi apparatus

• prostaglandin E2Reach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of prostaglandin E2 (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 7 routes

• N-benzoylglycineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. Hippurate (Benzoylglycinate) Formation

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

• 3-sulfino-L-alanineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:oxygen oxidoreductase

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

• (2E)-tetradecenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. trans-tetradec-2-enoyl-CoA reductase

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 8 morecytosol, mitochondrial matrixOne of 5 routes

• (2E)-decenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. trans-dec-2-enoyl-CoA reductase

  Catalysed by CPT1A, CPT1C, CPT1B, ACSM4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

• L-cysteinylglycineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by CPT1A, CPT1C, CPT1B, GGTLC3 and 5 morecytosol, mitochondrial matrixOne of 6 routes

• Fe(II)-heme aReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

  Catalysed by PTGS1, PTGS2, COX15endoplasmic reticulum, cytosol

• (2E)-hexenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. medium-chain acyl-CoA ligase (hexanoic acid)
  3. trans-hex-2-enoyl-CoA reductase

  Catalysed by CPT1A, CPT1C, CPT1B, ACSM4 and 6 morecytosol, mitochondrial matrixOne of 3 routes

• a cardiolipinReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + an acyl-CoA = a cardiolipin + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 4 morecytosol, Golgi apparatus

• (2E)-octenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. trans-oct-2-enoyl-CoA reductase

  Catalysed by CPT1A, CPT1C, CPT1B, CROT and 1 morecytosol, mitochondrial matrixOne of 5 routes

• 3-oxotetradecanoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. dodecanoyl-CoA + malonyl-CoA + H(+) = 3-oxotetradecanoyl-CoA + CO2 + CoA

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

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by SLC6A14, CLTRN, SLC6A20, PYCR1 and 2 moreextracellular space, cytosolOne of 4 routes

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by SLC6A14, SLC26A6, SLC4A1extracellular space, cytosolOne of 2 routes

• an all-trans-retinyl esterReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. all-trans-retinol + an acyl-CoA = an all-trans-retinyl ester + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 7 morecytosol, Golgi apparatusOne of 4 routes

• 1-O-hexadecyl-sn-glycero-3-phosphocholineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 4 morecytosol, Golgi apparatus

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

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 7 morecytosol, Golgi apparatus

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

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 9 morecytosol, Golgi apparatus

• a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-acyl-sn-glycero-3-phospho-(1'-sn-glycerol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

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

• sn-glycero-3-phospho-(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. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol) + H2O = sn-glycero-3-phospho-(1'-sn-glycerol) + (9Z)-octadecenoate + H(+)

  Catalysed by ABHD12endoplasmic reticulum, cytosolOne of 2 routes

• 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphateReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate + hexadecanoyl-CoA = 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphate + CoA

  Catalysed by CPT2, CPT1A, CPT1B, AGPAT3 and 1 moreperoxisomeOne of 2 routes

• 1-acyl-2-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. a 1-acyl-sn-glycero-3-phosphate + hexadecanoyl-CoA = 1-acyl-2-hexadecanoyl-sn-glycero-3-phosphate + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPCAT1peroxisome

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

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

• 3-oxoeicosasphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + L-serine + H(+) = 3-oxoeicosasphinganine + CO2 + CoA

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

• N-hexadecanoyl-(4R)-hydroxysphinganineReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. (4R)-hydroxysphinganine + hexadecanoyl-CoA = N-hexadecanoyl-(4R)-hydroxysphinganine + CoA + H(+)

  Catalysed by CPT2, CPT1A, CPT1B, CERS6 and 1 moreperoxisome

• 3-oxoeicosanoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. very-long-chain 3-oxoacyl-CoA synthase (malonyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 13 morecytosolOne of 2 routes

• 5-oxo-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate = 5-oxo-(6E,8Z,11Z,14Z)-eicosatetraenoate + H2O

  Catalysed by ALOX5, CYP1A1, CYP1A2, CYP1B1 and 1 moreendoplasmic reticulum, cytosol

• N-docosanoylsphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + sphinganine = N-docosanoylsphinganine + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 10 morecytosol

• N-eicosanoylsphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + sphinganine = N-eicosanoylsphinganine + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 10 morecytosol

• N-(octadecanoyl)-sphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. sphinganine + octadecanoyl-CoA = N-(octadecanoyl)-sphinganine + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 12 morecytosol

• N-hexadecanoylsphinganineReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. sphinganine + hexadecanoyl-CoA = N-hexadecanoylsphinganine + CoA + H(+)

  Catalysed by CPT2, CPT1A, CPT1B, CERS6 and 2 moreperoxisome

• N-(tetradecanoyl)-sphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. sphinganine + tetradecanoyl-CoA = N-(tetradecanoyl)-sphinganine + CoA + H(+)

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

• all-trans-retinyl 9Z-octadecenoateReach from here →
  1. Utilized Transport
  2. 1,2-di-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+) = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O
  3. 1,2,3-tri-(9Z-octadecenoyl)-glycerol + all-trans-retinol = all-trans-retinyl 9Z-octadecenoate + di-(9Z)-octadecenoylglycerol

  Catalysed by DGAT1, LIPA, PNPLA4, LIPE and 1 moreendoplasmic reticulum, cytosol

• 3-oxohexadecasphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + L-serine + H(+) = 3-oxohexadecasphinganine + CO2 + CoA

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

• 3-oxotetradecasphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. dodecanoyl-CoA + L-serine + H(+) = 3-oxotetradecasphinganine + CO2 + CoA

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

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by SLC6A14, NOS3, NOS1, NOS2extracellular space, cytosol

• Mo-molybdopterinReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by CPT1A, CPT1C, CPT1B, MOCOScytosol, mitochondrial matrixOne of 2 routes

• 3-oxooctadecanoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitate:CoA ligase (AMP-forming)
  3. very-long-chain 3-oxoacyl-CoA synthase (malonyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 14 morecytosolOne of 3 routes

• 3-oxodocosanoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. very-long-chain 3-oxoacyl-CoA synthase (eicosanoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 14 morecytosolOne of 3 routes

• 3-oxo-(8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(8Z,11Z,14Z)-eicosatrienoyl-CoA + CO2 + CoA

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

• 3-oxo-(8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (stearidonic acid)
  3. (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoA + CO2 + CoA

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

• (R)-noradrenalineReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of noradrenaline (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 4 routes

• 1-dodecanoyl-sn-glycerol 3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. glycerol-3-phosphate 1-O-acyltransferase (lauroyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 11 morecytosolOne of 2 routes

• 1-tetradecanoyl-sn-glycerol 3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. glycerol-3-phosphate 1-O-acyltransferase (myristoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 11 morecytosol

• 1-O-hexadecyl-2-hexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-O-hexadecyl-sn-glycero-3-phosphocholine + hexadecanoyl-CoA = 1-O-hexadecyl-2-hexadecanoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPCAT1peroxisome

• 1,2-dihexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + hexadecanoyl-CoA = 1,2-dihexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LCLAT1 and 2 moreperoxisome

• 1-tetradecanoyl-2-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-tetradecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + hexadecanoyl-CoA = 1-tetradecanoyl-2-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPGAT1peroxisome

• 1-octadecanoyl-2-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-octadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + hexadecanoyl-CoA = 1-octadecanoyl-2-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPGAT1peroxisome

• 1-(9Z-octadecenoyl)-2-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol) + hexadecanoyl-CoA = 1-(9Z-octadecenoyl)-2-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPGAT1peroxisomeOne of 2 routes

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

  Catalysed by ABHD16Aendoplasmic reticulum, cytosol