(S)-carnitine

Three steps

• N-hexacosanoylsphinganineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

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

• O-propanoyl-(R)-carnitineReach from here →
  1. solute carrier (L-carnitine)
  2. O-octanoyl-(R)-carnitine(in) + (R)-carnitine(out) = O-octanoyl-(R)-carnitine(out) + (R)-carnitine(in)
  3. propanoyl-CoA + (R)-carnitine = O-propanoyl-(R)-carnitine + CoA

  Catalysed by SLC25A20, CRATmitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• 3,3',5-triiodo-L-thyronineReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of S-glutathionyl-2-4-dinitrobenzene (cytosol to extracellular)

  Catalysed by SLCO1B1, SLCO1A2, SLCO1B3endoplasmic reticulum, cytosol, extracellular spaceOne of 8 routes

• 1-O-hexadecyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholineReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate
  3. 1-O-hexadecyl-sn-glycero-3-phosphocholine + (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA = 1-O-hexadecyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + CoA

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

• malonaldehydeReach from here →
  1. Utilized Transport
  2. prostaglandin-endoperoxide synthase (arachidonate)
  3. thromboxane A synthase (12(S)-HHT)

  Catalysed by PTGS1, PTGS2, TBXAS1endoplasmic reticulum, cytosolOne of 2 routes

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

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

• succinyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  3. (R)-methylmalonyl-CoA = succinyl-CoA

  Catalysed by CPT1A, CPT1C, CPT1B, ALDH6A1 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (3S)-3-hydroxybutanoyl-CoAReach from here →
  1. transport of butyryl-CoA and L-carnitine (cytosol to mitochondria)
  2. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
  3. (S)-3-hydroxybutanoyl-CoA hydro-lyase

  Catalysed by SLC33A1, ESCO2, OXSM, ACADS and 2 moremitochondrial matrix, cytosolOne of 2 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

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

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

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

• (2E)-glutaconyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by CPT1A, CPT1C, CPT1B, ACOX1cytosol

• propanoyl-CoAReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. (S)-methylmalonyl-CoA hydrolase
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by CPT1A, CPT1C, CPT1B, ECHDC1cytosol, mitochondrial matrix

• prostaglandin E1Reach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of prostaglandin E1 (cytosol to extracellular)

  Catalysed by SLCO1B1endoplasmic reticulum, cytosol, extracellular spaceOne of 4 routes

• prostaglandin I2Reach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of prostaglandin I2 (cytosol to extracellular)

  Catalysed by SLCO1B1endoplasmic reticulum, cytosol, extracellular spaceOne of 6 routes

• prostaglandin F2alphaReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by SLCO1B1endoplasmic reticulum, cytosol, extracellular spaceOne of 6 routes

• prostaglandin D2Reach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of prostaglandin D2 (cytosol to extracellular)

  Catalysed by SLCO1B1endoplasmic reticulum, cytosol, extracellular spaceOne of 7 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. Utilized Transport
  2. arachidonate:oxygen 15-oxidoreductase
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by ALOX15B, ALOX15, GPX5, GPX1 and 7 moreendoplasmic reticulum, cytosolOne of 4 routes

• 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate
  3. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate = 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoate + H2O

  Catalysed by ALOX15B, ALOX15, ALOX12, CYP1A1 and 6 moreendoplasmic reticulum, cytosolOne of 2 routes

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

• sarcosineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

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

• S-adenosyl 3-(methylsulfanyl)propylamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 2 morecytosol

• thromboxane A2Reach from here →
  1. Utilized Transport
  2. prostaglandin-endoperoxide synthase (arachidonate)
  3. thromboxane A synthase (prostaglandin H2)

  Catalysed by PTGS1, PTGS2, TBXAS1endoplasmic reticulum, cytosolOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, BAAT and 1 morecytosolOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

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

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by SLC6A14, ASLextracellular space, cytosolOne of 2 routes

• (R)-S-lactoylglutathioneReach from here →
  1. Utilized Transport
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by GLO1, GLOD4endoplasmic reticulum, cytosol, extracellular spaceOne of 2 routes

• propane-1,3-diamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 2 morecytosol

• carnosineReach from here →
  1. Carnitine Transport by Atb0
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC6A14, SLC6A6, CARNS1extracellular space, cytosol

• 4-guanidinobutanoateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by SLC6A14, GATMextracellular space, cytosolOne of 2 routes

• L-dopaReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitate:CoA ligase (AMP-forming)
  3. glycerol-3-phosphate 1-O-acyltransferase (palmitoyl-CoA)

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

• a 1-acylglycerone 3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

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

• (R)-2-hydroxyoctadecanoateReach from here →
  1. Utilized Transport
  2. Fas180
  3. octadecanoate + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (R)-2-hydroxyoctadecanoate + 2 Fe(III)-[cytochrome b5] + H2O

  Catalysed by FA2Hendoplasmic reticulum, cytosol

• 3-guanidinopropanoateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by SLC6A14, GATMextracellular space, cytosolOne of 2 routes

• L-histidineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• ethanolamineReach from here →
  1. Utilized Transport
  2. fatty acylamide amidohydrolase
  3. N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-ethanolamine + H2O = ethanolamine + (5Z,8Z,11Z,14Z)-eicosatetraenoate

  Catalysed by FAAH, FAAH2endoplasmic reticulum, cytosol

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

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 4 morecytosol, Golgi apparatusOne of 2 routes

• an N-acylglycineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 6 morecytosol, Golgi apparatusOne of 2 routes

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:2-oxoglutarate aminotransferase

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

• S-formylglutathioneReach from here →
  1. Utilized Transport
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by ADH1C, ADH1A, ADH5, ADH6 and 2 moreendoplasmic reticulum, cytosol, extracellular space

• succinate semialdehydeReach from here →
  1. Carnitine Transport by Atb0
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate

  Catalysed by SLC6A14, SLC6A12, ABATextracellular space, cytosol

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

• N-sulfo-D-glucosamineReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by SLC6A14, SLC26A6, SGSHextracellular space, cytosol

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

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

• dehydroepiandrosterone 3-sulfateReach from here →
  1. transport of butyryl-CoA and L-carnitine (cytosol to mitochondria)
  2. butanoyl-CoA + H2O = butanoate + CoA + H(+)
  3. dehydroepiandrosterone 3-sulfate(in) + butanoate(out) = dehydroepiandrosterone 3-sulfate(out) + butanoate(in)

  Catalysed by SLC33A1, ESCO2, OXSM, ACOT8 and 6 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• L-dopaquinoneReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by SLC6A14, TYRextracellular space, cytosol

• glutathioneReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

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

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

  Catalysed by CPT1A, CPT1C, CPT1B, SLC7A10cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 8 routes

• sphinganine 1-phosphateReach from here →
  1. Utilized Transport
  2. 2 H+[c] + NADH[c] + palmitate[c] = H2O[c] + hexadecanal[c] + NAD+[c]
  3. Sphinganine-1-phosphate pamlmitaldehyde-lyase

  Catalysed by ALDH3B1, ALDH3B2, ALDH3A2, SGPL1endoplasmic reticulum, cytosol

• L-saccharopineReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by SLC6A14, AASSextracellular space, cytosol, mitochondrial matrix