L-leucine

Three steps

• (2E,9Z)-octadecadienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. medium-chain acyl-CoA dehydrogenase (oleoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, ACADM and 3 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,9Z,12Z)-octadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosol

• (2E,4Z,7Z,10Z,13Z,16Z,19Z)-docosaheptaenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + O2 = (2E,4Z,7Z,10Z,13Z,16Z,19Z)-docosaheptaenoyl-CoA + H2O2

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• a fatty acyl-CoAReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATL2extracellular space, cytosol

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (9S)-hydroperoxy-(10E,12Z)-octadecadienoate + 2 glutathione = (9S)-hydroxy-(10E,12Z)-octadecadienoate + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, GPX1, GPX4extracellular space, cytosolOne of 2 routes

• (9R)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9R)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS1, PTGS2extracellular space, cytosol

• N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• 9-cis-retinolReach from here →
  1. Acetylation of Leucine
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 9-cis-retinol

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 2 moremitochondrial matrix, cytosol

• 18-hydroxy-(9Z)-octadecenoateReach from here →
  1. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate
  2. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate
  3. (9Z)-octadecenoate + reduced [NADPH--hemoprotein reductase] + O2 = 18-hydroxy-(9Z)-octadecenoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by PM20D1, CYP4A11, CYP4F11

• ursodeoxycholateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and ursodeoxycholate (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 2 routes

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

  Catalysed by PM20D1, ABHD12

• (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• tricosanoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (tricosanoic acid)
  3. Tricosanoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol

• 3-phenyllactateReach from here →
  1. transport of leucine and phenylalanine (cytosol to extracellular)
  2. Phenylalanine Transaminase, Mitochondrial
  3. Formation of Phenyl-Lactate

  Catalysed by SLC7A5, GOT2, TATextracellular space, cytosol, mitochondrial matrix

• prostaglandin G2Reach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin G2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 2 routes

• cholesteryl octadecanoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• N-octadecanoylhexadecasphinganineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. hexadecasphinganine + octadecanoyl-CoA = N-octadecanoylhexadecasphinganine + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphate = 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphate + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• 1-hexadecanoyl-2-eicosanoyl-sn-glycero-3-phosphocholineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-eicosanoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, GPX1, GPX2 and 1 moreextracellular space, cytosol

• sphinga-4E,14Z-dienineReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = sphinga-4E,14Z-dienine + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moremitochondrial matrix, Golgi apparatus

• 1',3'-bis-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerolReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol + (9Z,12Z)-octadecadienoyl-CoA = 1',3'-bis-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• a 1-acyl-2-octadecanoyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + octadecanoyl-CoA = a 1-acyl-2-octadecanoyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• cholesteryl (9Z)-hexadecenoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (palmitolate)
  3. (9Z)-hexadecenoyl-CoA + cholesterol = cholesteryl (9Z)-hexadecenoate + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• (9Z,12Z,15Z-octadecatrienoyl)-cholesterolReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (linolenate)
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + cholesterol = (9Z,12Z,15Z-octadecatrienoyl)-cholesterol + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• cholesteryl (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol, endoplasmic reticulum

• O-tetradecanoyl-(R)-carnitineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• O-octadecanoyl-(R)-carnitineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. (R)-carnitine + octadecanoyl-CoA = O-octadecanoyl-(R)-carnitine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• O-eicosanoyl-(R)-carnitineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + (R)-carnitine = O-eicosanoyl-(R)-carnitine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• O-4,8-dimethylnonanoyl-(R)-carnitineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by SLC6A14, CRAT, CROTextracellular space, cytosol, peroxisomeOne of 7 routes

• 1-octadecanoyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. 2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholine + octadecanoyl-CoA = 1-octadecanoyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• 1-octadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholineReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. 2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + octadecanoyl-CoA = 1-octadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterolReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• 2-glyceryl-prostaglandin H2Reach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 2-glyceryl-prostaglandin G2 + AH2 = 2-glyceryl-prostaglandin H2 + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• (2E,5Z)-tetradecadienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (physeteric acid)
  3. medium-chain acyl-CoA dehydrogenase (5-tetradecenoyl-CoA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• (2E,7Z)-hexadecadienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)
  3. medium-chain acyl-CoA dehydrogenase (7-hexadecenoyl-CoA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• 3-methylmalateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)

  Catalysed by BCAT2, AADAT, CLYBLmitochondrial matrix

• cholesteryl (11Z)-octadecenoateReach from here →
  1. Acetylation of Leucine
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix
  3. (11Z)-octadecenoyl-CoA + cholesterol = cholesteryl (11Z)-octadecenoate + CoA

  Catalysed by CPT1A, CPT1C, CPT1B, SOAT1mitochondrial matrix, cytosol

• (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, PTGES, MGST3 and 4 moreextracellular space, cytosol

• Se-methylselenocysteineReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. S-Adenosyl-L-methionine:thiol S-methyltransferase

  Catalysed by SLC7A5, SCLYextracellular space, cytosol

• (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, GPX1, GPX4extracellular space, cytosol

• thromboxane B2Reach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and thromboxane B2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO1B1extracellular space, cytosolOne of 2 routes

• a 3-oxoacyl-CoAReach from here →
  1. Acetylation of Leucine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + acetyl-CoA = a 3-oxoacyl-CoA + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moremitochondrial matrix, Golgi apparatus

• an S-substituted glutathioneReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by SLC7A5, GSS, GSTT4, GSTA4 and 21 moreextracellular space, cytosolOne of 2 routes

• prostaglandin H1Reach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. prostaglandin G1 + AH2 = prostaglandin H1 + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• (5S,15R)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,15R)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• (5S,15S)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,15S)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol

• 17R-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + AH2 + O2 = 17R-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + A + H2O

  Catalysed by SLC7A5, MOCOS, PTGS2extracellular space, cytosol