L-isoleucine

Two steps

• (9Z)-hexadecenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 11 routes

• L-cysteinylglycineReach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. gamma-glutamyltransferase (alanine)

  Catalysed by SLC7A5, GGTLC3, GGT1, GGT5 and 3 moreextracellular space, cytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by CPT1A, CPT1Bmitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 9 routes

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. transport of arginine and isoleucine (cytosol to extracellular)
  2. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by SLC7A6, NOS3, NOS1, NOS2extracellular space, cytosol

• Mo-molybdopterinReach from here →
  1. transport of alanine and isoleucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC7A5, MOCOSextracellular space, cytosol

• (2E)-octadecenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Carnitine O-Palmitoyltransferase

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (stearidonic acid)

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

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

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

• (15Z)-tetracosenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (ximenic acid)

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

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by SLC7A5, AGXT2extracellular space, cytosolOne of 2 routes

• nonadecanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Activation of dodecanedioic acid

  No catalyst recordedmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Production of Sebacoylcarnitine

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Production of Suberylcarnitine

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Activation of adipic acid for Formation of Adipoyl Carnitine

  No catalyst recordedmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by ACAA1, HSD17B4, EHHADH, ACOX1mitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Production of Hexadecanedioylcarnitine

  Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. fatty-acid-CoA ligase

  Catalysed by SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• thio-Mo-molybdopterinReach from here →
  1. transport of cysteine and isoleucine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC7A5, MOCOSextracellular space, cytosol

• glycodeoxycholateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. Glycodeoxycholate amidohydrolase

  Catalysed by SLC7A5extracellular space, cytosolOne of 2 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. long-chain-fatty-acid-CoA ligase (physeteric acid)

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

• (3E)-decenoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by ACADM, ECHS1, ACAA2, HADHmitochondrial matrix

• N-tetradecanoylglycineReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATL2extracellular space, cytosol

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. Acetylation of Isoleucine
  2. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by HADHA, ACAA2, HADHBmitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by SLC7A5, BAATextracellular space, cytosol

• N-docosanoylglycineReach from here →
  1. transport of glycine and isoleucine (cytosol to extracellular)
  2. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by SLC7A5, BAATextracellular space, cytosol