(R)-carnitine

Three steps

• L-aspartateReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A13inner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• formic acidReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. transport of betaine and chloride (cytosol to extracellular)
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by SLC22A5, SLC6A12, SLC26A4inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• malonic acidReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. transport of betaine and chloride (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by SLC22A5, SLC6A12inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• tetradecanoateReach from here →
  1. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA
  2. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA
  3. Myristoyl-CoA hydrolase

  Catalysed by CPT2, ACOT7, BAAT, THEM4 and 2 morecytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. Utilized Transport

  Catalysed by CPT2, CPT1A, CPT1Bperoxisome, endoplasmic reticulum, cytosolOne of 3 routes

• pentanoateReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. estrone 3-sulfate(out) + glutarate(in) = estrone 3-sulfate(in) + glutarate(out)
  3. pentanoate(in) + estrone 3-sulfate(out) = pentanoate(out) + estrone 3-sulfate(in)

  Catalysed by SLC22A8, SLC22A24, SLC22A11, SLC22A9inner side of the membrane, outer side of the membraneOne of 4 routes

• octadecan-1-olReach from here →
  1. (R)-carnitine + octadecanoyl-CoA = O-octadecanoyl-(R)-carnitine + CoA
  2. (R)-carnitine + octadecanoyl-CoA = O-octadecanoyl-(R)-carnitine + CoA
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

  Catalysed by CPT2, FAR1, FAR2

• eicosanoateReach from here →
  1. eicosanoyl-CoA + (R)-carnitine = O-eicosanoyl-(R)-carnitine + CoA
  2. eicosanoyl-CoA + (R)-carnitine = O-eicosanoyl-(R)-carnitine + CoA
  3. Eicosanoyl-CoA hydrolase

  Catalysed by CPT2, BAAT, ACOT6, ACOT1cytosolOne of 4 routes

• (9Z)-hexadecenoateReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. Utilized Transport

  Catalysed by CPT2, CPT1A, CPT1Bperoxisome, endoplasmic reticulum, cytosolOne of 6 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. Utilized Transport

  Catalysed by CPT2, CPT1A, CPT1Bperoxisome, endoplasmic reticulum, cytosolOne of 2 routes

• nicotinateReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. nicotinate(in) + urate(out) = nicotinate(out) + urate(in)

  Catalysed by SLC22A8, SLC22A12, SLC22A13inner side of the membrane, outer side of the membrane

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC22A5, BHMT, BHMT2, IL4I1inner side of the membrane, outer side of the membrane, cytosol

• 1-O-hexadecyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholineReach from here →
  1. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. 1-O-hexadecyl-sn-glycero-3-phosphocholine + (9Z)-octadecenoyl-CoA = 1-O-hexadecyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by CPT2, LPCAT4

• cholesteryl hexadecanoateReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by CPT2, CPT1A, CPT1B, SOAT2 and 1 moreperoxisome, endoplasmic reticulumOne of 2 routes

• (S)-lactic acidReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. urate(in) + glutarate(out) = urate(out) + glutarate(in)
  3. transport of L-lactate and urate (cytosol to extracellular)

  Catalysed by SLC22A8, SLC22A12inner side of the membrane, outer side of the membrane, extracellular space, cytosol

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by SLC22A8, SUGCTinner side of the membrane, outer side of the membraneOne of 3 routes

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. cholesterol + (9Z)-octadecenoyl-CoA = cholesteryl (9Z-octadecenoate) + CoA

  Catalysed by CPT2, SOAT2, SOAT1

• L-ornithineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. transport of methionine and ornithine (cytosol to extracellular)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A6inner side of the membrane, outer side of the membrane, cytosol, extracellular spaceOne of 2 routes

• taurineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. transport of betaine and chloride (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC22A5, SLC6A12, SLC6A6inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• 1,2-di-(9Z-octadecenoyl)-glycerolReach from here →
  1. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. 1-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoyl-CoA = 1,2-di-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CPT2, DGAT1, MOGAT2, AWAT2 and 3 moreOne of 2 routes

• 1,2-di-(9Z-octadecenoyl)-sn-glycerolReach from here →
  1. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. 2-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoyl-CoA = 1,2-di-(9Z-octadecenoyl)-sn-glycerol + CoA

  Catalysed by CPT2, DGAT1, MOGAT3, DGAT2

• 1,2,3-tri-(9Z-octadecenoyl)-glycerolReach from here →
  1. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. 1,2-di-(9Z-octadecenoyl)-sn-glycerol + (9Z)-octadecenoyl-CoA = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CPT2, DGAT1, MOGAT2, AWAT1 and 5 moreOne of 3 routes

• succinyl-CoAReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. glutarate + succinyl-CoA = glutaryl-CoA + succinate

  Catalysed by SLC22A8, SUGCTinner side of the membrane, outer side of the membrane

• glycineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. transport of glycine and methionine (cytosol to extracellular)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A5inner side of the membrane, outer side of the membrane, cytosol, extracellular spaceOne of 8 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. dodecanoyl-CoA + (R)-carnitine = O-dodecanoyl-R-carnitine + CoA
  2. carnitine O-palmitoyltransferase (dodecanoylcarnitine)
  3. trans-dodec-2-enoyl-CoA reductase

  Catalysed by CPT2, CPT1A, CPT1C, CPT1B and 1 morecytosol, mitochondrial matrixOne of 5 routes

• (2E)-butenoyl-CoAReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)

  Catalysed by SLC22A8, SUGCT, GCDHinner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA
  2. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

  Catalysed by CPT2, ELOVL6, ELOVL3

• (2E)-glutaconyl-CoAReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by SLC22A8, SUGCT, ACOX1inner side of the membrane, outer side of the membrane

• butanoyl-CoAReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. transport of butyryl-CoA and L-carnitine (cytosol to mitochondria)

  Catalysed by CPT2, CPT1A, CPT1B, SLC33A1 and 2 moreperoxisome, mitochondrial matrix, cytosolOne of 2 routes

• itaconyl-CoAReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate

  Catalysed by SLC22A8, SUGCTinner side of the membrane, outer side of the membraneOne of 5 routes

• 15-oxoprostaglandin E2Reach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. prostaglandin E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)
  3. prostaglandin E2 + NAD(+) = 15-oxoprostaglandin E2 + NADH + H(+)

  Catalysed by SLC22A8, HPGDinner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin H2Reach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. prostaglandin E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)
  3. prostaglandin E synthase (prostaglandin E2)

  Catalysed by SLC22A8, PTGES, PTGES3, PTGES2inner side of the membrane, outer side of the membrane, endoplasmic reticulumOne of 4 routes

• prostaglandin D2Reach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. prostaglandin F2alpha(out) + glutarate(in) = prostaglandin F2alpha(in) + glutarate(out)
  3. aldo-keto reductase (prostaglandin D2)

  Catalysed by SLC22A8, AKR1C3inner side of the membrane, outer side of the membrane, cytosol

• sarcosineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by SLC22A5, BHMT, DMGDHinner side of the membrane, outer side of the membrane

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

  Catalysed by CPT2, CPT1A, CPT1B, GPAT3 and 3 moreperoxisome, cytosolOne of 2 routes

• guanidinoacetateReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. transport of betaine and chloride (cytosol to extracellular)
  3. Transport of Guanidinoacetic Acid

  Catalysed by SLC22A5, SLC6A12inner side of the membrane, outer side of the membrane, extracellular space, cytosol

• L-tryptophanReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. transport of methionine and tryptophan (cytosol to extracellular)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A5inner side of the membrane, outer side of the membrane, cytosol, extracellular spaceOne of 6 routes

• glutathioneReach from here →
  1. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  2. taurocholate(out) + glutarate(in) = taurocholate(in) + glutarate(out)
  3. taurocholate(in) + glutathione(in) + ATP + H2O = taurocholate(out) + glutathione(out) + ADP + phosphate + H(+)

  Catalysed by SLC22A8, SLC22A24, ABCC4inner side of the membrane, outer side of the membraneOne of 3 routes

• beta-alanineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. transport of betaine and chloride (cytosol to extracellular)
  3. transport of beta-alanine and chloride (cytosol to extracellular)

  Catalysed by SLC22A5, SLC6A12, SLC6A6, SLC6A14inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• L-alanineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. transport of alanine and methionine (cytosol to extracellular)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A5inner side of the membrane, outer side of the membrane, cytosol, extracellular spaceOne of 9 routes

• L-asparagineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. transport of asparagine and methionine (cytosol to extracellular)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A5inner side of the membrane, outer side of the membrane, cytosol, extracellular spaceOne of 5 routes

• L-phenylalanineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. transport of methionine and phenylalanine (cytosol to extracellular)

  Catalysed by SLC22A5, BHMT, BHMT2, SLC7A5inner side of the membrane, outer side of the membrane, cytosol, extracellular spaceOne of 8 routes

• L-homocysteineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC22A5, BHMT, BHMT2inner side of the membrane, outer side of the membrane, cytosol

• S-methyl-L-methionineReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC22A5, BHMT, BHMT2inner side of the membrane, outer side of the membrane, cytosol

• a 1-acyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholineReach from here →
  1. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. a 1-acyl-sn-glycero-3-phosphocholine + (9Z)-octadecenoyl-CoA = a 1-acyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by CPT2, LPCAT4

• 3-oxosphinganineReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

  Catalysed by CPT2, CPT1A, CPT1B, SPTLC1 and 2 moreperoxisome, cytosolOne of 2 routes

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. glyceronephosphate O-acyltransferase (DHAP)

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

• an acyl-CoAReach from here →
  1. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  2. palmitoyl-CoA:L-carnitine O-palmitoyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by CPT2, CPT1A, CPT1Bperoxisome, cytosolOne of 5 routes

• L-methionine (S)-S-oxideReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC22A5, BHMT, BHMT2, MSRAinner side of the membrane, outer side of the membrane, cytosol

• L-methionine (R)-S-oxideReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC22A5, BHMT, BHMT2, MSRB3 and 2 moreinner side of the membrane, outer side of the membrane, cytosol