(S)-carnitine

Three steps

• 2-[12-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl]-glycerolReach from here →
  1. Utilized Transport
  2. fatty acid amide hydrolase (2-arachidonoylglycerol)
  3. 2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-glycerol + O2 = 2-[12-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl]-glycerol

  Catalysed by FAAH, ALOX15Bendoplasmic reticulum, cytosol

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Glutamine by Atb0 Transporter
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by SLC6A14, PFASextracellular space, cytosol

• kinetensinReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Chymase

  Catalysed by SLC6A14, CMA1extracellular space, cytosolOne of 3 routes

• N-hexadecanoyl-sphinga-4E,14Z-dienineReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. sphinga-4E,14Z-dienine + hexadecanoyl-CoA = N-hexadecanoyl-sphinga-4E,14Z-dienine + CoA + H(+)

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

• an N-(fatty acyl)-glycineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

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

• 1-(10Z-heptadecenoyl)-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphoethanolamineReach from here →
  1. Utilized Transport
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA = 1-(10Z-heptadecenoyl)-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphoethanolamine + CoA

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

• 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamine + CoA

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

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 8 morecytosolOne of 2 routes

• cholesteryl (7Z)-octadecenoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. (7Z)-octadecenoyl-CoA + cholesterol = cholesteryl (7Z)-octadecenoate + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, SOAT1cytosol

• (15S)-15-hydroperoxy-prostaglandin E2Reach from here →
  1. Utilized Transport
  2. Arachidonate:oxygen oxidoreductase
  3. prostaglandin G2 = (15S)-15-hydroperoxy-prostaglandin E2

  Catalysed by PTGS1, PTGS2, PTGESendoplasmic reticulum, cytosol

• acetaldehydeReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Threonine by Atb0 Transporter
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by SLC6A14extracellular space, cytosol

• acetylcholineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Acetyl-CoA:choline O-acetyltransferase
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by CPT1A, CPT1C, CPT1B, CHATcytosol, nucleusOne of 7 routes

• pyruvateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by CPT1A, CPT1C, CPT1B, AGXT2cytosol, mitochondrial matrixOne of 26 routes

• all-trans-retinoic acidReach from here →
  1. transport of butyryl-CoA and L-carnitine (cytosol to mitochondria)
  2. Butyryl-CoA hydrolase
  3. transport of butyrate and retinoate (cytosol to extracellular)

  Catalysed by SLC33A1, ESCO2, OXSM, ACOT12 and 2 moremitochondrial matrix, cytosol, extracellular spaceOne of 6 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)

  Catalysed by SLC6A14, PAHextracellular space, cytosolOne of 3 routes

• CHEBI:15467Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Acetoacetate:CoA ligase (AMP-forming)
  3. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, AACS and 1 morecytosolOne of 3 routes

• CHEBI:15570Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 4 morecytosol, mitochondrial matrix, extracellular spaceOne of 5 routes

• (S)-malateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. ATP citrate lyase (citrate)
  3. transport of malate and OAA (cytosol to mitochondria)

  Catalysed by CPT1A, CPT1C, CPT1B, ACLYcytosol, mitochondrial matrixOne of 9 routes

• CHEBI:15611Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

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

• a 1-hexadecanoyl-2-acylglycerolReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. a 2-acylglycerol + hexadecanoyl-CoA = a 1-hexadecanoyl-2-acylglycerol + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPGAT1peroxisome

• an hexadecanoyl-acylglycerolReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. a 1-acylglycerol + hexadecanoyl-CoA = an hexadecanoyl-acylglycerol + CoA

  Catalysed by CPT2, CPT1A, CPT1B, LPGAT1peroxisome

• 1-(10Z-heptadecenoyl)-2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamineReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z)-octadecenoyl-CoA = 1-(10Z-heptadecenoyl)-2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine + CoA

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

• 1-(10Z-heptadecenoyl)-2-hexadecanoyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + hexadecanoyl-CoA = 1-(10Z-heptadecenoyl)-2-hexadecanoyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by CPT2, CPT1A, CPT1B, MBOAT1peroxisome

• 10-formyltetrahydrofolic acidReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and formate (cytosol to extracellular)
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by SLC6A14, SLC26A4, MTHFD1extracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC6A14, PAHextracellular space, cytosolOne of 2 routes

• S-(9Z-octadecenoyl)-4'-phosphopantetheineReach from here →
  1. Utilized Transport
  2. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate
  3. (9Z)-octadecenoyl-CoA + H2O = S-(9Z-octadecenoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• S-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-4'-phosphopantetheineReach 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 + H2O = S-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• CHEBI:15699Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and homoserine (cytosol to extracellular)

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 3 morecytosol, mitochondrial matrix, extracellular spaceOne of 52 routes

• betaine aldehydeReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Acetyl-CoA:choline O-acetyltransferase
  3. choline dehydrogenase (choline)

  Catalysed by CPT1A, CPT1C, CPT1B, CHAT and 1 morecytosol, nucleusOne of 4 routes

• trimethylenediamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by CPT1A, CPT1C, CPT1B, SAT1 and 2 morecytosolOne of 3 routes

• butanalReach from here →
  1. transport of butyryl-CoA and L-carnitine (cytosol to mitochondria)
  2. butanoyl-CoA + H2O = butanoate + CoA + H(+)
  3. butanal + NAD(+) + H2O = butanoate + NADH + 2 H(+)

  Catalysed by SLC33A1, ESCO2, OXSM, ACOT8 and 6 moremitochondrial matrix, cytosol

• CHEBI:15765Reach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by SLC6A14, THextracellular space, cytosolOne of 5 routes

• malonateReach from here →
  1. Carnitine Transport by Atb0
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. malonate(in) + sulfate(out) = malonate(out) + sulfate(in)

  Catalysed by SLC6A14, SLC26A6, SLC25A14, SLC25A30 and 1 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 1-acylglycerone 3-phosphateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:glycerone-phosphate O-acyltransferase

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

• bromideReach from here →
  1. Utilized Transport
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by MGST3, GSTA4, GSTA1, GSTA2 and 13 moreendoplasmic reticulum, cytosol, extracellular spaceOne of 2 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. Carnitine Transport by Atb0
  2. Transport of L-Glutamine by Atb0 Transporter
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by SLC6A14, GFPT2, GFPT1extracellular space, cytosol

• phosphatidylethanolamineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase

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

• 11-cis-retinalReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol:NAD+ oxidoreductase

  Catalysed by CPT1A, CPT1C, CPT1B, DGAT1 and 11 morecytosolOne of 4 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

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

• cholesterolReach from here →
  1. Utilized Transport
  2. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate
  3. beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4E-enine + cholesterol = N-(9Z-octadecenoyl)-sphing-4-enine + cholesteryl 3-beta-D-glucoside

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moreendoplasmic reticulum, cytosol

• hexadecan-1-olReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitate:CoA ligase (AMP-forming)
  3. formate dehydrogenase (palmitoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 9 morecytosol, peroxisomeOne of 2 routes

• ureaReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by SLC6A14, ARG1extracellular space, cytosolOne of 3 routes

• 11-cis-retinyl hexadecanoateReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by CPT1A, CPT1C, CPT1B, SLC27A2 and 12 morecytosolOne of 4 routes

• succinic semialdehydeReach from here →
  1. Carnitine Transport by Atb0
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate:2-oxoglutarate aminotransferase

  Catalysed by SLC6A14, SLC6A12, ABATextracellular space, cytosol, mitochondrial matrix

• L-cystineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by CPT1A, CPT1C, CPT1B, SLC7A9 and 1 morecytosol, mitochondrial matrix, extracellular spaceOne of 7 routes

• (R)-3-aminoisobutyric acidReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by CPT1A, CPT1C, CPT1B, AGXT2cytosol, mitochondrial matrix

• lithocholic acidReach from here →
  1. Utilized Transport
  2. transport of arachidonate (cytosol to extracellular)
  3. transport of lithocholate (cytosol to extracellular)

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

• phosphatidic acidReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1-acyl-sn-glycerol-3-phosphate 2-O-acyltransferase

  Catalysed by CPT1A, CPT1C, CPT1B, ASAH1 and 12 morecytosol, Golgi apparatus, mitochondrial matrixOne of 7 routes

• L-citrullineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of citrulline and glycine (cytosol to extracellular)

  Catalysed by CPT1A, CPT1C, CPT1B, GLYAT and 5 morecytosol, mitochondrial matrix, extracellular spaceOne of 39 routes

• nitric oxideReach from here →
  1. Carnitine Transport by Atb0
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by SLC6A14, NOS3, NOS1, NOS2extracellular space, cytosolOne of 2 routes