itaconate

Three steps

• hexanedioyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrix, cytosolOne of 7 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 2 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CRAT and 1 moremitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACAA1 and 3 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Production of Hexadecanedioylcarnitine

  Catalysed by SUCLG1, SUCLG2, SUCLA2, CPT1A and 2 moremitochondrial matrix, cytosolOne of 2 routes

• O-dodecanoyl-R-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. dodecanoyl-CoA + (R)-carnitine = O-dodecanoyl-R-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• pristanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. fatty-acid-CoA ligase

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 3 routes

• O-tetradecanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-octadecanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. (R)-carnitine + octadecanoyl-CoA = O-octadecanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-eicosanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. eicosanoyl-CoA + (R)-carnitine = O-eicosanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• (5Z)-tetradecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• O-(9Z)-octadecenoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2inner side of the membrane, outer side of the membrane

• O-4,8-dimethylnonanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. 4,8-dimethylnonanoyl-CoA + (R)-carnitine = O-4,8-dimethylnonanoyl-(R)-carnitine + CoA

  Catalysed by SUGCT, SLC22A8, CPT2, CRAT and 1 moreinner side of the membrane, outer side of the membrane

• (3E)-decenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by SUCLG1, SUCLG2, SUCLA2, ACADM and 3 moremitochondrial matrix

• O-hexanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. hexanoyl-CoA + (R)-carnitine = O-hexanoyl-(R)-carnitine + CoA

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

• O-3-oxobutanoyl-(R)-carnitineReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. acetoacetyl-CoA + (R)-carnitine = O-3-oxobutanoyl-(R)-carnitine + CoA

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

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, HADHA and 2 moremitochondrial matrix

• 16alpha-hydroxydehydroepiandrosterone 3-sulfateReach from here →
  1. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate
  2. dehydroepiandrosterone 3-sulfate(out) + glutarate(in) = dehydroepiandrosterone 3-sulfate(in) + glutarate(out)
  3. dehydroepiandrosterone 3-sulfate + reduced [NADPH--hemoprotein reductase] + O2 = 16alpha-hydroxydehydroepiandrosterone 3-sulfate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by SUGCT, SLC22A24, SLC22A8, CYP3A7inner side of the membrane, outer side of the membrane

• (7Z)-hexadecenoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. Itaconate:CoA ligase (GDP-forming)
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• a 3-oxoacyl-CoAReach from here →
  1. Itaconate:CoA ligase (GDP-forming)
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. a 3-oxo acid + succinyl-CoA = a 3-oxoacyl-CoA + succinate

  Catalysed by SUCLG1, SUCLG2, SUCLA2, SUGCT and 2 moremitochondrial matrix