L-leucine

Three steps

• spermine monoaldehydeReach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. primary-amine oxidase (spermine)

  Catalysed by SAT1, SAT2, AOC2, AOC1 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• O-octanoyl-(R)-carnitineReach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. solute carrier (L-carnitine)

  Catalysed by SLC6A14, SLC25A20extracellular space, cytosol, mitochondrial matrixOne of 11 routes

• CHEBI:18132Reach from here →
  1. transport of chloride and leucine (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. ATP:choline phosphotransferase

  Catalysed by SLC6A14, SLC5A7, CHKA, CHKBextracellular space, cytosol

• CHEBI:18183Reach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by SLC7A5, GGT1, GGT5, GGTLC2 and 5 moreextracellular space, cytosolOne of 4 routes

• dodecanoateReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosolOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by SLC7A5, GARTextracellular space, cytosol

• arsenic triglutathioneReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by SLC7A5, GSS, AS3MTextracellular space, cytosolOne of 3 routes

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. Acetylation of Leucine
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by SAT1, SAT2, AOC2, AOC1 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• CHEBI:18405Reach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by SLC7A5, SCLY, PNPOextracellular space, cytosolOne of 3 routes

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

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

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by SLC7A5, AGXT2, GCLCextracellular space, cytosol

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

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

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. transport of leucine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by SLC7A5, SDS, SDSL, BCKDHA and 1 moreextracellular space, cytosol, mitochondrial matrix

• N-methyltryptamineReach from here →
  1. transport of leucine and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase
  3. S-Adenosyl-L-methionine:amine N-methyltransferase

  Catalysed by SLC7A5, DDC, INMTextracellular space, cytosol

• D-isoleucineReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membrane

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-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. 15-deoxy-Delta(12,14)-prostaglandin J2 + glutathione = 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathione

  Catalysed by SLC7A5, GSS, MGST3extracellular space, cytosol

• Xaa-L-Pro dipeptideReach from here →
  1. transport of alanine and leucine (cytosol to extracellular)
  2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)
  3. Xaa-L-Pro dipeptide + H2O = an L-alpha-amino acid + L-proline

  Catalysed by SLC7A5, SLC1A4, PEPDextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 11-cis-retinyl esterReach from here →
  1. Acetylation of Leucine
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 1 moremitochondrial matrix, cytosol

• (6R)-10-formyltetrahydrofolateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by SLC7A5, SHMT1, SHMT2, MTHFD1 and 1 moreextracellular space, cytosol

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

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

• (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

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

• (15S)-hydroxy-(11Z,13E)-eicosadienoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (15S)-hydroperoxy-(11Z,13E)-eicosadienoate + 2 glutathione = (15S)-hydroxy-(11Z,13E)-eicosadienoate + glutathione disulfide + H2O

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

• (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (17S)-hydroperoxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + 2 glutathione = (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + glutathione disulfide + H2O

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

• a hydroxy-1,2-diacyl-glycero-3-phosphocholineReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxy-1,2-diacyl-glycero-3-phosphocholine + 2 glutathione = a hydroxy-1,2-diacyl-glycero-3-phosphocholine + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, GPX4extracellular space, cytosol

• glycodeoxycholate 3-sulfateReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. Glycodeoxycholate amidohydrolase
  3. glycodeoxycholate + 3'-phosphoadenylyl sulfate = glycodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLC7A5, SULT2A1extracellular space, cytosol

• 3',5'-diiodo-L-thyronineReach 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. 3',5'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5'-triiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by BCAT2, AADAT, AGXT2mitochondrial matrixOne of 10 routes

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by BCAT2, AADAT, AGXT2mitochondrial matrixOne of 4 routes

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. L-Leucine:2-oxoglutarate aminotransferase
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. N(omega)-methyl-L-arginine + glyoxylate = 5-(3-methylguanidino)-2-oxopentanoate + glycine

  Catalysed by BCAT2, AADAT, AGXT2mitochondrial matrixOne of 4 routes

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Acetylation of Leucine
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 4 moremitochondrial matrix, cytosol

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Acetylation of Leucine
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CROT, MOGAT2, MOGAT1mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. Acetylation of Leucine
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• (6R)-5,10-methylenetetrahydrofolic acidReach from here →
  1. transport of glycine and leucine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by SLC7A5, SHMT1extracellular space, cytosolOne of 7 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. Acetylation of Leucine
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 3 moremitochondrial matrix, cytosol

• 3-iodothyronamineReach 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. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 2 routes

• 3-iodo-L-thyronineReach 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. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 2 routes

• 3'-iodo-L-thyronineReach 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. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, ELOVL7 and 6 moremitochondrial matrix, cytosol

• L-thyronineReach 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. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• thyronamineReach 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. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• 3'-iodothyronamineReach 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. 3'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• 3,3'-diiodothyronamineReach 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. 3,3'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• 3',5'-diiodothyronamineReach 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. 3',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• 3,3',5'-triiodothyronamineReach 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. 3,3',5'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• L-homocysteine thiolactoneReach from here →
  1. transport of leucine and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP = L-homocysteine thiolactone + AMP + diphosphate

  Catalysed by SLC3A2, SLC7A5, BHMT2, MARS1extracellular space, cytosol

• L-homocysteinyl-AMPReach from here →
  1. transport of leucine and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP + H(+) = L-homocysteinyl-AMP + diphosphate

  Catalysed by SLC3A2, SLC7A5, BHMT2, MARS1extracellular space, cytosol

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

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by HADHA, ACADVL, HADHB, ACADL and 1 moremitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by HADHA, ACADVL, HADHBmitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Acetylation of Leucine
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by HADHA, ACADVL, HADHB, ACADL and 1 moremitochondrial matrix